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CURRICULUM VITAE AND BIBLIOGRAPHY December 2, 2016 NAME: Mark Alan Knepper POSITION: Principal Investigator, Epithelial Systems Biology Laboratory, Division of Intramural Research, NHLBI, NIH, Bethesda, MD CURRENT ADDRESS:

Epithelial Systems Biology Laboratory Building 10, Room 6N307 MSC-1603 National Institutes of Health Bethesda, MD 20892-1603 Phone: (301) 496-3064 FAX: (301) 402-1443 Electronic mail: knep@helix.nih.gov Web Page: http://esbl.nhlbi.nih.gov/ POSITIONS: 2009- : Principal Investigator, Epithelial Systems Biology Laboratory, Division of Intramural Research (DIR), National Heart, Lung and Blood Institute (NHLBI), NIH, Bethesda, MD 2010-2011: Acting Director, Systems Biology Center, DIR, NHLBI, NIH 2002-2009: Chief, Laboratory of Kidney and Electrolyte Metabolism, NHLBI, DIR, NIH 1988-2009: Chief, Renal Mechanisms Section, Laboratory of Kidney and Electrolyte Metabolism, NHLBI, DIR, NIH 1984-2009: Senior Investigator, Laboratory of Kidney and Electrolyte Metabolism, NHLBI, NIH EDUCATION: M.D., Case Western Reserve University, Cleveland, Ohio, 1976. Frackelton Research Award. Ph.D., Biomedical Engineering, Case Western Reserve University, Cleveland, Ohio, 1975. Doctoral Thesis: "Systems Modeling and Experimental Studies on Renal Urea, Sodium, Chloride, and Water Transport." B.S.E., summa cum laude, Chemical Engineering, University of Michigan, Ann Arbor, Michigan, 1970. (Received Singleton Award, 1968 [outstanding sophomore in College of Engineering]; Distinguished Scholar Award in 1970 [presented to the engineering senior with the highest academic grade point average])

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ACADEMIC POSITIONS: 1989-2003: Visiting Associate Professor, Division of Nephrology, Department of Medicine, Johns Hopkins University, Baltimore, MD 1977-1978: Teaching Fellow in Medicine, Department of Medicine, Boston University School of Medicine, Boston, Massachusetts. 1975-1976: Research Associate, Department of Biomedical Engineering, Case Western Reserve University, Cleveland, OH POST GRADUATE TRAINING:

1982-1984: Medical Staff Fellow, Laboratory of Kidney and Electrolyte Metabolism, NHLBI, NIH, Bethesda, Maryland 1978-1982: PHS Staff Associate, Laboratory of Kidney and Electrolyte Metabolism, NHLBI, NIH Bethesda, Maryland. 1976-1978: Intern and Resident in Medicine, University Hospital, Boston, Massachusetts. UNIFORMED SERVICES:

United States Public Health Services, Corps of Commissioned Officers -Active Duty: 1978-1982, 1984-2008. PHS #45361, Regular Corps -Rank: Captain (Naval Equivalent) Retired.

BOARD CERTIFICATION:

American Board of Internal Medicine (General Internal Medicine), 1980 PROFESSIONAL SOCIETY MEMBERSHIPS:

American Heart Association (Council on the Kidney in Cardiovascular Disease, Council on High Blood Pressure Research)

American Physiological Society American Association for the Advancement of Science American Society of Nephrology Biomedical Engineering Society

RESEARCH INTERESTS:

Systems Biology: Mathematical modeling of physiological systems; large-scale data integration Proteomics: Antibody- and mass spectrometry-based methods, applied to kidney and urine Physiology: Renal transport mechanisms; regulation of renal water and NaCl excretion Pathophysiology: Water and sodium balance disorders

Bioengineering: Development of ultramicro-analytical methods and specialized software for interpretation of proteomic data HONORARY SOCIETIES:

Tau Beta Pi Phi Kappa Phi Phi Lambda Upsilon Alpha Chi Sigma

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AWARDS AND HONORS: 1990: PHS Commendation Medal 1995: PHS Achievement Medal 1996: Carl W. Gottschalk Distinguished Lectureship of the American Physiological Society (Experimental Biology '96 Meeting, Washington, DC, April, 1996) 1997: Fellow of the American Association for the Advancement of Science (February 15, 1997) 1997: Robert W. Berliner Lecturer, Yale University, February 27, 1997 1999: Fellow, American Institute for Medical and Biological Engineering 2001: Homer W. Smith Award, American Society of Nephrology (October 15, 2001) 2004: Principal Plenary Lecturer, Australia-New Zealand Society of Nephrology Annual Meeting, September, 2004. 2006: Clarenburg Lectureship, Kansas State University, November, 2006 2008: Donald W. Seldin Lectureship of the American Heart Association (Council on the Kidney

in Cardiovascular Disease), September, 2008. 2008: Distinguished Alumnus Award, Case Western Reserve University School of Medicine,

October, 2008. 2009: Honorary Doctorate, Aarhus University, Denmark, September 11, 2009. 2012: Hugh Davson Distinguished Lectureship of the American Physiological Society (Experimental Biology '12 Meeting, San Diego, CA, April, 2012)

2013: Si-Ki Hong Memorial Lecture, Department of Physiology & Biophysics, School of Medicine, University of Buffalo/SUNY, May, 2013.

2104: Robert W. Berliner Award for Excellence in Renal Physiology, American Physiological Society (Experimental Biology ’14 Meeting, San Diego, CA, April, 2014)

2104: Plenary Speaker, Korean Society of Nephrology Annual Meeting, May, 2014. 2105: Invited Speaker, 8th Federation of Asian and Oceanian Physiological Societies, Annual Meeting, November, 2015, Bangkok, Thailand COMMITTEE MEMBERSHIPS (Intramural, Selected): 1985-1999 : Animal Care and Use Committee; NHLBI; Intramural Program 1995-1997 : Central Facilities Advisory Committee, NIH Intramural Program 1999-2011 : Promotions and Tenure Committee, NHLBI; Intramural Program

2002-2009 : Chairman, Proteomics Core Users Committee, NHLBI 2006-2007 : Chairman, Bioinformatics-Systems Biology Core Users Committee, NHLBI 2006- : Electron Microscopy Core Users Committee, NHLBI 2007- : DNA Microarray Core Users Committee, NHLBI

2007-2008 : Member, NHLBI Basic Biomedical Research Tenure Track Search Committee 2008-2009 : Member, NHLBI Basic Biomedical Research Tenure Track Search Committee 2009-2010 : Member, NHLBI Basic Biomedical Research Tenure Track Search Committee 2009-2010 : Member, Trans-NIH Systems Biology Tenure Track Search Committee 2009- : Member, DNA Sequencing Core Users Committee, NHLBI 2009 : Chair, Tenure/Tenure Track Search Committee, NIDCR 2010- : Member, Computer Scientist Review Panel (advisory to the NIH Central Tenure Committee). 2010-2011 : Chair, Systems Biology Search Committee, Pan-NIH Stadtman Investigator Search

2011-2012: Chair, Search Committee for Director of NHLBI Bioinformatics/Computational Biology Core Facility

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2013- : Chair, Bioinformatics and Systems Biology Core Steering Committee, DIR, NHLBI

2013- : Information Technology Steering Committee, DIR, NHLBI 2015- : Chair, Bioinformatics Core Director, Search Committee 2015 : Co-Chair, Earl Stadtman NIH-wide Tenure Track Search, Systems

Biology Subcommittee COMMITTEE MEMBERSHIPS (Extramural):

1986-1990: American Heart Association: Cardiorenal Study Committee 1989-1992: Program Committee, American Physiological Society, Renal Section

Representative 1990: Program Committee, American Society of Nephrology 1990: Ad hoc Member, General Medicine B Study Section, Division of

Research Grants, NIH 1991-1994: Regular Member, General Medicine B Study Section, Division of

Research Grants, NIH 1996-1999: Chairman, Renal Section of the American Physiological Society 1998-2001: Member, Renal Commission, International Union of Physiological

Sciences 2003 : Member, NHLBI Working Group on Clinical Proteomics for Heart, Lung, & Blood Diseases, May 8-9, 2003 2002-2005: Member, Publications Committee, American Physiological Society 2006- : Member, Steering Committee, HUPO Urine and Kidney Initiative 2007- : Member, Steering Committee, Cell and Molecular Physiology Section, American

Physiological Society 2010 : Participant, NIDDK Urine Biospecimen Workshop, February 22-23, 2010

EDITORIAL POSITIONS:

1989-2003: Editorial Board, American Journal of Physiology: Renal Physiology 1990-1996: Editorial Board, Journal of the American Society of Nephrology 1993-2006: Editorial Board, Seminars in Nephrology 1996-2001: Associate Editor, Journal of the American Society of Nephrology 1998-2002: Board of Consulting Editors, Journal of Clinical Investigation 2002-2008 : Editorial Board, Journal of Clinical Investigation

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DUTIES OF CURRENT POSITION (Public Health Service Job Description):

1. Direction of research program of Epithelial Systems Biology Laboratory, NHLBI, NIH 2. Long term planning of research program aimed at developing necessary physiological

knowledge for understanding of water and NaCl balance disorders 3. Recruitment and training of personnel; career guidance 4. Design of specialized instrumentation for renal research 5. Development of novel methodologies for study of renal function 6. Management of research budget 7. Design of individual scientific experiments 8. Conduct of and interpretation of scientific experiments 9. Development of appropriate mathematical models for design and interpretation of physiological experiments 10. Writing and research reporting 11. Education of renal researchers in academic community about methods available and appropriate for individual research programs 12. Development of online databases and web-based tools for renal research 12. Direction of staff in all of above activities

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BIBLIOGRAPHY 1. Knepper, M. A., R.A. Danielson, G.M. Saidel and K.H. Johnston: Effects of dietary protein restriction and glucocorticoids on urea excretion in rats. Kidney Int. 8:303-315, 1975. 2. Knepper, M.A., G.M. Saidel and P.J. Palatt: Mathematical model of renal regulation of urea excretion. Med. Biol. Eng. 14:408-426, 1976. 3. Knepper, M.A., G.M. Saidel and R.A. Danielson: Simulation of tubular mass transport using perfectly mixed compartments. Proc. 1976 Summer Computer Simulation Conference, pp 468-472, 1976. 4. Knepper, M.A., C.V. Gunter and R.A. Danielson: Effects of glucagon on renal function in protein-deprived rats. Surg. Forum 29-31, 1977. 5. Knepper, M.A., R.A. Danielson, G.M. Saidel and R.S. Post: Quantitative analysis of renal medullary anatomy in rats and rabbits. Kidney Int. 12:313-323, 1977. 6. Saidel, G.M., P.S. Chandhoke and M.A. Knepper: Spatially discrete models of counter-current mass transport for application to the kidney. Mathematics and Computers in Simulation 20:259-270, 1978. 7. Garg, L.C., M.A. Knepper and M.B. Burg: Mineralocorticoid effects on sodium and potassium adenosine triphosphatase in individual nephron segments. Am. J. Physiol. 240:F536-F544, 1981. 8. Knepper, M.A. and M.B. Burg: Increased fluid absorption and cell volume in isolated rabbit proximal straight tubules following in vivo DOCA administration. Am. J. Physiol. 241:F502-F508, 1981. 9. Knepper, M.A.: Measurement of osmolality in kidney slices using vapor pressure osmometry. Kidney Int. 21:653-655, 1982. 10. Vurek, G.G. and M.A. Knepper: A colorimeter for measurement of picomole quantities of urea. Kidney Int. 2l:656-658, 1982. 11. Proud, D., M.A. Knepper and J.J. Pisano: Distribution of immunoreactive kallikrein along the rat nephron. Am. J. Physiol. 244:F5l0-F5l5, 1983. 12. Knepper, M.A.: Urea transport in nephron segments from medullary rays of rabbits. Am. J. Physiol. 244:F622-F627, 1983. 13. Knepper, M.A. and M.B. Burg: Organization of nephron function. Am. J. Physiol. 244:F579-F589, 1983. 14. Balaban, R.S. and M.A. Knepper: Nitrogen-l4 nuclear magnetic resonance spectroscopy of mammalian tissues. Am. J. Physiol. 245:C439-C444, 1983. 15. Knepper, M.A.: Urea transport in isolated thick ascending limbs and collecting ducts from rats. Am. J. Physiol. 245:F634-F639, 1983.

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16. Good, D.W., M.A. Knepper and M.B. Burg: Ammonia and bicarbonate transport by thick ascending limb of rat kidney. Am. J. Physiol. 247:F35-F44, 1984. 17. Knepper, M.A., D.W. Good and M.B. Burg: Mechanism of ammonia secretion by cortical collecting ducts of rabbits. Am. J. Physiol. 247: F729-F738, 1984. 18. Good, D. and M. Knepper: Ammonia transport in the mammalian kidney. Am. J. Physiol. 248: F459-F471, 1985. 19. Tomita, K., J.J. Pisano and M.A. Knepper: Control of sodium and potassium transport in the cortical collecting duct of the rat. Effects of bradykinin, vasopressin, and deoxycorticosterone. J. Clin. Invest. 76: 132-136, 1985. 20. Garcia-Austt, J., D.W. Good, M.B. Burg and M.A. Knepper: Deoxycorticosterone-stimulated bicarbonate secretion in rabbit cortical collecting ducts: effects of luminal chloride removal and in vivo acid loading. Am. J. Physiol. 249: F205-F212, 1985. 21. Good, D.W., M.A. Knepper and M.B. Burg: Ammonia absorption by the thick ascending limb of Henle's loop. Contr. Nephrol. 47: 110-115, 1985. 22. Knepper, M.A., D.W. Good and M.B. Burg: Mechanism of ammonia secretion by rabbit cortical collecting ducts. Contr. Nephrol. 47: 116-124, 1985. 23. Star, R.A., M.B. Burg and M.A. Knepper: Bicarbonate secretion and chloride absorption by rabbit cortical collecting ducts. J. Clinical Invest. 76: 1123-1130, 1985. 24. Chandhoke, P.S., G.M. Saidel and M.A. Knepper: Role of inner medullary collecting duct NaCl transport in urinary concentration. Am. J. Physiol. 249: F688-F697, 1985. 25. Garvin, J.L., M.B. Burg and M.A. Knepper: Ammonium replaces potassium in supporting sodium transport by the Na-K-ATPase of renal proximal straight tubules. Am. J. Physiol. 249: F785-F788, 1985. 26. Knepper, M.A., D.W. Good and M.B. Burg: Ammonia and bicarbonate transport by rat cortical collecting ducts perfused in vitro. Am. J. Physiol. 249: F870-F877, 1985. 27. Tomita, K., J.J. Pisano, M.B. Burg, and M.A. Knepper: Effects of vasopressin and bradykinin on anion transport by the rat cortical collecting duct. Evidence for an electroneutral sodium chloride transport pathway. J. Clin. Invest. 77:136-141, 1986. 28. Knepper, M.A. and J.L. Stephenson: Urinary concentrating and diluting processes (Chapter 39). In: Physiology of Membrane Disorders. T.E. Andreoli, D.D. Fanestil, J.F. Hoffman, and S.G. Schultz, eds. Second edition. New York, Plenum, 1986. 29. Burg, M.B. and M.A. Knepper: Single tubule perfusion techniques. Kidney Int. 30:166-170, 1986. 30. Sands, J.M., M.A. Knepper, and K.R. Spring: Na-K-Cl cotransport in apical membrane of rabbit

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renal papillary surface epithelium. Am. J. Physiol. 251: F475-F484, 1986. 31. Kurtz, I., R. Star, R.S. Balaban, J.L. Garvin, and M.A. Knepper: Spontaneous luminal disequilibrium pH in S3 proximal tubules. Role in ammonia and bicarbonate transport. J. Clin. Invest. 78: 989-996, 1986. 32. Sands, J.M., and M.A. Knepper: Urea permeability of mammalian inner medullary collecting duct system and papillary surface epithelium. J. Clin. Invest. 79: 138-147, 1987. 33. Knepper, M.A., and F. Roch-Ramel: Pathways of urea transport in the mammalian kidney. Kidney Int. 31: 629-633, 1987. 34. Nonoguchi, H., M.A. Knepper, and V.C. Manganiello: Effects of atrial natriuretic factor on cyclic guanosine monophosphate and cyclic adenosine monophosphate accumulation in microdissected nephron segments from rats. J. Clin. Invest. 79: 500-507, 1987. 35. Garvin, J.L., M.B. Burg, and M.A. Knepper: NH3 and NH4

+ transport by rabbit renal proximal straight tubules. Am. J. Physiol. 252: F232-F239, 1987. 37. Star, R.A., M.B. Burg, and M.A. Knepper: Luminal pH disequilibrium and ammonia transport in outer medullary collecting duct. Am. J. Physiol. 252: F1148-F1157, 1987. 38. Knepper, M.A., M.B. Burg, J. Orloff, R.W. Berliner, and F.C. Rector, Jr.: Ammonium, urea, and systemic pH regulation (letter). Am. J. Physiol. 253: F199-F200, 1987. 39. Garvin, J.L. and M.A. Knepper: Bicarbonate and ammonia transport in isolated perfused rat proximal straight tubules. Am. J. Physiol. 253: F277-F281, 1987. 40. Sands, J.M., H. Nonoguchi, and M.A. Knepper: Vasopressin effects on urea and H2O transport in inner medullary collecting duct subsegments. Am. J. Physiol. 253: F823-F832, 1987. 41. Star, R.A., I. Kurtz, R. Mejia, M.B. Burg, and M.A. Knepper. Disequilibrium pH and ammonia transport in isolated perfused cortical collecting ducts. Am. J. Physiol. 253: F1232-F1242, 1987. 42. Star, R.A., H. Nonoguchi, R. Balaban, and M.A. Knepper: Calcium and cyclic adenosine monophosphate as second messengers for vasopressin in the rat inner medullary collecting duct. J. Clin. Invest. 81: 1879-1888, 1988. 43. Garvin, J.L., M.B. Burg, and M.A. Knepper: Active NH4

+ absorption by the thick ascending limb. Am. J. Physiol. 255: F57-F65, 1988. 44. Knepper, M.A. and M.B. Burg: Renal Acid-base Transport (Chapter 6). In: Diseases of the Kidney. R.W. Schrier and C.W. Gottschalk, eds. Fourth edition. Boston, Little Brown, 1988, pp. 211-239. 45. Knepper, M.A.: Renal Transport of Ammonium in Systemic pH Regulation (Chapter 14). In: pH Homeostasis: Mechanisms and Control. D. Haussinger, ed. London, Academic Press, 1988, pp. 305-322.

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46. Knepper, M.A., J.M. Sands, H. Nonoguchi, R.A. Star, and R.K. Packer: Inner Medullary Collecting Duct. In: Nephrology. A.M. Davison, ed. London, Bailliere Tindall, 1988, pp. 317-331. 47. Sands, J.M., H. Nonoguchi, and M.A. Knepper. Regulation of Urea and Water Transport by Vasopressin in Renal Inner Medullary Collecting Ducts (Chapter 16). In: Vasopressin: Cellular and Integrative Functions. A.W. Cowley, J.-F. Liard, and D.A. Ausiello, eds. New York, Raven Press, 1988, pp. 137-142. 48. Sands, J.M., H. Nonoguchi, and M.A. Knepper. Hormone effects on NaCl permeability of rat inner medullary collecting duct. Am. J. Physiol. 255: F421-F428, 1988. 49. Nonoguchi, H., J.M. Sands, and M.A. Knepper. Atrial natriuretic factor inhibits vasopressin-stimulated osmotic water permeability in rat inner medullary collecting duct. J. Clin. Invest. 82: 1383-1390, 1988. 50. Knepper, M.A., R. Packer, and D.W. Good: Ammonium transport in the kidney. Physiol. Reviews 69: 179-249, 1989. 51. Nonoguchi, H., J.M. Sands, and M.A. Knepper. ANF inhibits NaCl and fluid absorption in cortical collecting duct of rat kidney. Am. J. Physiol. 256: F179-F186, 1989. 52. Terada, Y. and M.A. Knepper: Na+-K+-ATPase activities in renal tubule segments of rat inner medulla. Am. J. Physiol. 256: F218-F223, 1989. 53. Sands, J.M., Y. Terada, L.M. Bernard, and M.A. Knepper. Aldose reductase activities in microdissected rat renal tubule segments. Am. J. Physiol. 256: F563-F569, 1989. 54. Knepper, M.A., J.M. Sands, and C.-L. Chou. Independence of urea and water transport in rat inner medullary collecting duct. Am. J. Physiol. 256: F610-F621, 1989. 55. Brown, J.C., R.K. Packer, and M.A. Knepper. Role of organic anions in the renal response to dietary acid and base loads. Am. J. Physiol. 257: F170-F176, 1989. 56. Packer, R.K., J.M. Sands, and M.A. Knepper. Chloride and osmotic water permeabilities of isolated rabbit renal papillary surface epithelium. Am. J. Physiol. 257: F218-F224, 1989. 57. Chou, C.-L., and M.A. Knepper. Inhibition of urea transport in inner medullary collecting duct by phloretin and urea analogs. Am. J. Physiol. 257: F359-F365, 1989. 58. Knepper, M.A. Regulation of electrolyte transport in the renal cortical collecting duct by vasopressin and natriuretic peptides. In: Progress in Atrial Peptide Research (American Society of Hypertension Symposium Series, Volume 3). B.M. Brenner, and J.H. Laragh, eds. New York, Raven Press, 1989, pp. 95-109. 59. Terada, Y., and M.A. Knepper. Continuous-flow quantitation of Na+ and K+ in nanoliter samples using chromogenic macrocyclic ionophores. Am. J. Physiol. 257: F893-F898, 1989. 60. Mejia, R., J.M. Sands, J.L. Stephenson, and M.A. Knepper. Renal actions of atrial natriuretic

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factor: a mathematical modelling study. Am. J. Physiol. 257: F1146-F1157, 1989. 61. Wall, S.M., J.M. Sands, M.F. Flessner, H. Nonoguchi, K.R. Spring, and M.A. Knepper. Net acid transport by isolated perfused inner medullary collecting ducts. Am. J. Physiol. 258: F75-F84, 1990. 62. Wall, S.M., and M.A. Knepper. Acid-base transport in the inner medullary collecting duct. Sem. Nephrol. 10: 148-158, 1990. 63. Good, D.W., and M.A. Knepper. Mechanisms of ammonium excretion: Role of the renal medulla. Sem. Nephrol. 10: 166-173, 1990. 64. Chou, C.-L., J.M. Sands, H. Nonoguchi, and M.A. Knepper. Concentration dependence of urea/thiourea transport in rat inner medullary collecting duct. Am. J. Physiol. 258: F486-F494, 1990. 65. Chandhoke, P.S., R.K. Packer, and M.A. Knepper. Apical acidification by rabbit papillary surface epithelium. Am. J. Physiol. 258: F893-F899, 1990. 66. Knepper, M.A. Facilitated transport of urea in the renal inner medullary collecting duct. In: The Frontiers of Nephrology (Excerpta Medica International Congress Series). R.W. Berliner, N. Honda, and K.J. Ullrich, eds. Excerpta Medica, Amsterdam, 1990, pp. 237-246. 67. Mejia, R., and M.A. Knepper. Acid-base transport in the renal thick ascending limb. Math. Comp. Modelling 14: 538-542, 1990. 68. Chou, C.L., J.M. Sands, H. Nonoguchi, and M.A. Knepper. Urea-gradient associated fluid absorption with σurea=1 in rat terminal collecting duct. Am. J. Physiol. 258: F1173-F1180, 1990. 69. Wright, P.A., and M.A. Knepper. Glutamate dehydrogenase activities in microdissected rat nephron segments: The effects of acid-base loading. Am. J. Physiol. 259: F53-F59, 1990. 70. Knepper, M.A. and R.A. Star. The vasopressin-regulated urea transporter in renal inner medullary collecting duct. Am. J. Physiol. 259: F393-F401, 1990. 71. Terada, Y. and M.A. Knepper. Thiazide-sensitive NaCl absorption in rat cortical collecting duct. Am. J. Physiol. 259: F519-F528, 1990. 72. Flessner, M.F. and M.A. Knepper. Ammonium transport in collecting ducts. Min. Electr. Metab. 16: 299-307, 1990. 73. Wright, P.A., M.B. Burg, and M.A. Knepper. Microdissection of kidney tubule segments. In: Methods in Enzymology, S. Fleischer and E. Fleisher, eds., Academic Press, vol. 191, pp. 226-231, 1990. 74. Wright, P.A. and M.A. Knepper. Phosphate-dependent glutaminase activity in rat renal cortical and medullary tubule segments. Am. J. Physiol. 259: F961-F970, 1990. 75. Knepper, M.A., and F.C. Rector, Jr. Urinary Concentration and Dilution (Chapter 12). In: The Kidney, Brenner, B.M., and F.C. Rector, Jr., eds. Philadelphia, Saunders, 1991, 445-482.

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76. Flessner, M.F., S.M. Wall, and M.A. Knepper. Permeabilities of rat collecting duct segments to NH3 and NH4

+. Am. J. Physiol. 260: F264-F272, 1991. 77. Wall, S.W., M.F. Flessner, and M.A. Knepper. Distribution of luminal carbonic anhydrase activity along rat inner medullary collecting duct. Am. J. Physiol. 260: F738-F748, 1991. 78. Knepper, M.A. Ultramicro-analysis of tubular fluid by continuous-flow methodology. In: Nephrology, M. Hatano, ed. Tokyo: Springer-Verlag Tokyo, 1991, pp. 1438-1444. 79. Knepper, M.A. NH4

+ transport in the kidney. Kidney Int. 40: S95-S102, 1991. 80. Wall, S.M., C.-L. Chou, and M.A. Knepper. The vasopressin-regulated urea transporter in the terminal collecting duct of the mammalian kidney. In: Vasopressin, Jard, S., and R. Jamison, eds. London: Libbey, 1991, pp. 483-491. 81. Packer, R.K., S.S. Desai, K. Hornbuckle, and M.A. Knepper. Role of countercurrent multiplication in renal ammonium handling: Regulation of medullary ammonium accumulation. J. Am. Soc. Nephrology 2:77-83, 1991. 82. Lankford, S.P., C.-L. Chou, Y. Terada, S.M. Wall, J.B. Wade, and M.A. Knepper. Regulation of collecting duct water permeability independent of cAMP-mediated AVP response. Am. J. Physiol. 261: F554-F566, 1991. 83. Knepper, M.A., S.P. Lankford, and Y. Terada. Renal tubular actions of ANF. Can. J. Physiol. Pharmacol. 69: 1537-1545, 1991. 84. Wright, P.A., and M.A. Knepper. Mapping ammoniagenic enzyme activities along the rat nephron. Contrib. Nephrol. 92:130-135, 1991. 85. Knepper, M.A., S.S. Desai, K. Hornbuckle, R.K. Packer. Regulation of renal medullary ammonium accumulation. Contrib. Nephrol. 92:119-23, 1991. 86. Terada, Y., T. Moriyama, B.M. Martin, M.A. Knepper, and A. Garcia-Perez. PT-PCR microlocalization of mRNA for guanylyl cyclase-coupled ANF receptor in rat kidney. Am. J. Physiol. 261: F1080-F1087, 1991. 87. Flessner, M.F., S.M. Wall, and M.A. Knepper. Ammonium and bicarbonate transport in rat outer medullary collecting ducts. Am. J. Physiol. 262: F1-F7, 1992. 88. Wall, S.M., J.S. Han, C.-L. Chou, and M.A. Knepper. Kinetics of urea and water permeability activation by vasopressin in rat terminal IMCD. Am. J. Physiol. 262: F989-F998, 1992. 89. Wright, P.A., R.K. Packer, A. Garcia-Perez, and M.A. Knepper. Time course of renal glutamate dehydrogenase induction during NH4Cl loading in rats. Am. J. Physiol. 262: F999-F1006, 1992. 90. Marsh, D.J., and M.A. Knepper. Renal handling of urea. In: Handbook of Physiology: Renal Physiology, E. Windhager, ed., New York: Oxford Press, 1992, pp. 1317-1347.

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91. J.S. Han, K.A. Thompson, C.-L. Chou, and M.A. Knepper. Experimental tests of three-dimensional model of urinary concentrating mechanism. J. Am. Soc. Nephrol. 2: 1677-1688, 1992. 92. Y. Maeda, Y. Terada, H. Nonoguchi, and M.A. Knepper. Hormone and autocoid regulation of cyclic AMP production in rat IMCD subsegments. Am. J. Physiol. 263: F319-F327, 1992. 93. C.L. Chou and M.A. Knepper. In vitro perfusion of chinchilla thin limb segments: Segmentation and osmotic water permeability. Am. J. Physiol. 263: F417-F426, 1992. 94. M.A. Knepper, C.-L. Chou, and H. Layton. How is urine concentrated by the renal inner medulla? Contrib. Nephrol. 102:144-160, 1993. 95. Nielsen, S., B.L. Smith, E.I. Christensen, M.A. Knepper, and P. Agre. CHIP28 water channels are localized in constitutively water-permeable segments of the nephron. J. Cell. Biol. 120: 371-383, 1993. 96. Chou, C.L. and M.A. Knepper. In vitro perfusion of chinchilla thin limb segments: Urea and NaCl permeabilities. Am. J. Physiol. 264: F337-F343, 1993. 97. Knepper, M.A. and M.F. Flessner. Renal Acid-base Transport (Chapter 6). In: Diseases of the Kidney. R.W. Schrier and C.W. Gottschalk, eds. Fifth edition. Boston, Little Brown, 1993, pp. 207-232. 98. Flessner, M.F., R. Mejia, and M.A. Knepper. Ammonium and bicarbonate transport in isolated perfused rodent long-loop thin descending limb. Am. J. Physiol. 264: F388-F396, 1993. 99. Mejia, R., M.F. Flessner, and M.A. Knepper. Model of ammonium and bicarbonate transport along the long-loop thin descending limb: Implications for alkalinization of luminal fluid. Am. J. Physiol. 264: F397-F403, 1993. 100. Flessner, M.F. and M.A. Knepper. Ammonium and bicarbonate transport in isolated perfused ascending limbs of the loop of Henle. Am. J. Physiol. 264: F837-F844, 1993. 101. Chou, C.-L., M.A. Knepper, and H.E. Layton. Urinary concentrating mechanism: Role of the inner medulla. Seminars in Nephrology 13:168-181, 1993. 102. Maeda, Y., J.S. Han, C.C. Gibson, and M.A. Knepper. Vasopressin and oxytocin receptors coupled to Ca2+ mobilization in rat inner medullary collecting duct. Am. J. Physiol. 264: F15-F25, 1993. 103. Han, J.S., Y. Maeda, and M.A. Knepper. Dual actions of vasopressin and oxytocin in regulation of water permeability in terminal collecting duct. Am. J. Physiol. 264: F26-F34, 1993. 104. Nielsen, S., and M.A. Knepper. Vasopressin activates collecting duct urea transporters and water channels by distinct physical processes. Am. J. Physiol. 265: F204-F213, 1993. 105. Knepper, M.A., and S. Nielsen. Kinetic model of water and urea permeability regulation by vasopressin in collecting duct. Am. J. Physiol. 265: F214-F224, 1993.

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106. Nielsen, S., J. Muller, and M.A. Knepper. Vasopressin- and cAMP-induced changes in ultrastructure of isolated perfused inner medullary collecting ducts. Am. J. Physiol. 265: F225-F238, 1993. 107. Ostrowski, N.L., W.S. Young III, M.A. Knepper, and S.J. Lolait. Expression of vasopressin V1a and V2 receptor messenger ribonucleic acid in the liver and kidney of embryonic, developing, and adult rats. Endocrinology 133: 1849-1859, 1993. 108. Chou, C.-L., S. Nielsen, and M.A. Knepper. Structural-functional correlation in thin limbs of the chinchilla long-loop of Henle: Evidence for a novel papillary subsegment. Am. J. Physiol. 265: F863-F874, 1993. 109. S. Nielsen, S.R. DiGiovanni, E.I. Christensen, M.A. Knepper, and H.W. Harris. Cellular and subcellular immunolocalization of vasopressin-regulated water channel in rat kidney. Proc. Nat. Acad. Sci. USA 90: 11663-11667, 1993. 110. Knepper, M.A., J.S. Han, Y. Maeda, C. Ecelbarger, S. DiGiovanni, C.-L. Chou, S. Nielsen, and J.B. Wade. Vasopressin-independent regulation of collecting duct water permeability: Long-term and short-term factors. In: Vasopressin, Richter, D., and Gross, G., eds. Paris: Libbey, 1993, pp. 381-392. 111. Han J.S., Y. Maeda, C. Ecelbarger, and M.A. Knepper. Vasopressin-independent regulation of collecting duct water permeability. Am. J. Physiol. 266: F139-F146, 1994. 112. Wade, J.B., S. Nielsen, R.A. Coleman, and M.A. Knepper. Long-term regulation of collecting duct water permeability: Freeze-fracture analysis of isolated tubules. Am. J. Physiol. 266: F223-F230, 1994. 113. Knepper, M.A., M.F. Flessner, R. Mejia, and C.-L. Chou. NH4

+ and NH3 permeabilities of Henle's loop segments. Contrib. Nephrology 110: 60-66, 1994. 114. DiGiovanni, S.R., and M.A. Knepper. An RT-PCR approach to study the regulation of mRNA levels for phosphate-dependent glutaminase. Contrib. Nephrology 110: 81-87, 1994. 115. Knepper, M.A. The aquaporin family of molecular water channels (Commentary). Proc. Nat. Acad. Sci. USA 91: 6255-6258, 1994. 116. Knepper, M.A., S. Nielsen, C.-L. Chou, and S.R. DiGiovanni. Mechanism of vasopressin action in the renal collecting duct. Seminars in Nephrology 14: 302-321, 1994. 117. DiGiovanni, S.R., K.M. Madsen, A.D. Luther, and M.A. Knepper. Dissociation of ammoniagenic enzyme adaptation in rat S1 proximal tubules and ammonium excretion response. Am. J. Physiol. 267: F407-F414, 1994. 118. DiGiovanni, S.R., S. Nielsen, E.I. Christensen, and M.A. Knepper. Regulation of collecting duct water channel expression by vasopressin in Brattleboro rat. Proc. Nat. Acad. Sci. USA 91: 8984-8988, 1994. 119. Ecelbarger, C.E., Y. Maeda, C.C. Gibson, and M.A. Knepper. Extracellular ATP increases intracellular calcium in the rat terminal collecting duct via a nucleotide receptor. Am. J. Physiol. 267:

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F998-F1006, 1994. 120. Maeda, Y., B.L. Smith, P. Agre, and M.A. Knepper. Quantification of aquaporin-CHIP water channel protein in microdissected renal tubules by fluorescence-based ELISA. J. Clin. Invest. 95: 422-428, 1995. 121. Nielsen, S., C.-L. Chou, D. Marples, E.I. Christensen, B.K. Kishore, and M.A. Knepper. Vasopressin increases water permeability of kidney collecting duct by inducing translocation of aquaporin-CD water channels to plasma membrane. Proc. Nat. Acad. Sci. USA 92: 1013-1017, 1995. 122. Chou, C.-L., S.R. DiGiovanni, R. Mejia, S. Nielsen, and M.A. Knepper. Oxytocin as an antidiuretic hormone: I. Concentration dependence of action. Am. J. Physiol. 269: F70-F77, 1995. 123. Chou, C.-L., S.R. DiGiovanni, A. Luther, S.J. Lolait, and M.A. Knepper. Oxytocin as an antidiuretic hormone: II. Role of V2 vasopressin receptor. Am. J. Physiol. 269: F78-F85, 1995. 124. Marples, D., M.A. Knepper, E.I. Christensen, and S. Nielsen. Redistribution of aquaporin-2 water channels induced by vasopressin in rat kidney inner medullary collecting duct. Am. J. Physiol. 269: C655-C664, 1995. 125. Knepper, M.A., and C.-L. Chou. Urea and ammonium transport in the mammalian kidney. In: Nitrogen Metabolism and Excretion, Walsh, P.J., and Wright, P.A., eds. Boca Raton: CRC Press, 1995, pp. 187-208. 126. Smith, C.P., W.-S. Lee, S. Martial, M.A. Knepper, J.M. Sands, and M.A. Hediger. Cloning and regulation of expression of the rat kidney urea transporter (rUT2). J. Clin. Invest. 96: 1556-1563, 1995. 127. Nielsen, S., D. Marples, M. Mohtashami, N.O. Dalby, W. Trimble, and M. Knepper. Expression of VAMP2-like protein in kidney collecting duct intracellular vesicles: Co-localization with aquaporin-2 water channels. J. Clin. Invest. 96: 1834-1844, 1995. 128. Layton, H.E., E.B. Pitman, and M.A. Knepper. A dynamic numerical method for models of the urine concentrating mechanism. SIAM J. of. Appl. Math. 55; 1390-1418, 1995. 129. Ecelbarger, C.A., J. Terris, G. Frindt, M. Echevarria, D. Marples, S. Nielsen, and M.A. Knepper. Aquaporin-3 water channel localization and regulation in rat kidney. Am. J. Physiol. 269: F663-F672, 1995. 130. Terris, J., C.A. Ecelbarger, D. Marples, M.A. Knepper, and S. Nielsen. Distribution of aquaporin-4 water channel expression within rat kidney. Am. J. Physiol. 269: F775-F785, 1995. 131. Kishore, B.K., C.-L. Chou, and M.A. Knepper. Extracellular nucleotide receptor inhibits AVP-stimulated water permeability in inner medullary collecting duct. Am. J. Physiol. 269: F863-F869, 1995. 132. Nielsen, S., and M. Knepper. Vasopressin regulation of aquaporin 2 water channels in kidney collecting duct. In: Neurohypophysis: Recent Progress of Vasopressin and Oxytocin Research, Saito, T, K. Kurokawa, and S. Yoshida, eds. Amsterdam: Elsevier, 1995, pp. 473-483.

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133. Layton, H.E., M.A. Knepper, and C.-L. Chou. Permeability criteria for effective function of passive countercurrent multiplier. Am. J. Physiol. 270: F9-F20, 1996. 134. Knepper, M.A., and F.C. Rector, Jr. Urinary Concentration and Dilution (Chapter 13). In: The Kidney, Brenner, B.M., ed. Philadelphia, Saunders, 1996, pp. 532-570. 135. Ecelbarger, C.A., C.-L. Chou, S.J. Lolait, M.A. Knepper, and S.R. DiGiovanni. Evidence for dual signaling pathways for V2 receptor in rat inner medullary collecting duct. Am. J. Physiol. 270: F623-F633, 1996. 136. Marples, D., J. Frokiaer, J. Dorup, M.A. Knepper, and S. Nielsen. Hypokalemia-induced downregulation of aquaporin-2 water channel expression in rat kidney medulla and cortex. J. Clin. Invest. 97: 1960-1968, 1996. 137. Frokiaer, J., D. Marples, M.A. Knepper, and S. Nielsen. Bilateral ureteral obstruction downregulates expression of the vasopressin sensitive aquaporin-2 water channel in rat kidney. Am. J. Physiol. 270: F657-F668, 1996. 138. Elliot, S., P. Goldsmith, M. Knepper, M. Haughey, and B. Olson. Urinary excretion of aquaporin-2 in humans: A potential marker of collecting duct responsiveness to vasopressin. J. Am. Soc. Nephrol. 7: 403-409, 1996. 139. Knepper, M.A., J.B. Wade, J. Terris, C.A. Ecelbarger, D. Marples, B. Mandon, C.-L. Chou, B.K. Kishore, and S. Nielsen. Renal aquaporins. Kidney International 49: 1712-1717, 1996. 140. Nielsen S., D. Marples, M. Knepper, and P. Agre. The aquaporin family of water channels in kidney: An update on physiology and pathophysiology of aquaporin-2. Kidney International 49: 1718-1723, 1996. 141. Nielsen, S., J. Terris, C.P. Smith, M.A. Hediger, C.A. Ecelbarger, and M.A. Knepper. Cellular and subcellular localization of the vasopressin-regulated urea transporter in rat kidney. Proc. Natl. Acad. Sci. USA 93: 5495-5500, 1996. 142. Kishore, B.K., B. Mandon, N.B. Oza, S.R. DiGiovanni, R.A. Coleman, N.L. Ostrowski, J.B. Wade, and M.A. Knepper. Rat renal arcade segment expresses vasopressin-regulated water channel and vasopressin V2 receptor. J. Clin. Invest. 97: 2763-2771, 1996. 143. Devuyst, O., C.R. Burrow, B.L. Smith, P. Agre, M.A. Knepper, and P.D. Wilson. Expression of aquaporins 1 and 2 during nephrogenesis and in autosomal dominant polycystic kidney disease. Am. J. Physiol. 271: F169-F183, 1996. 144. Kishore, B.K., J.M. Terris, and M.A. Knepper. Quantitation of aquaporin-2 abundance in microdissected collecting ducts: Axial distribution and control by AVP. Am. J. Physiol. 271: F62-F70, 1996. 145. Terris, J., C.A. Ecelbarger, S. Nielsen, and M.A. Knepper. Long-term regulation of four renal aquaporins in rats. Am. J. Physiol. 271: F414-F422, 1996.

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146. Ecelbarger, C.A., J. Terris, J.R. Hoyer, S. Nielsen, J.B. Wade, and M.A. Knepper. Localization and regulation of the rat renal Na+-K+-2Cl- cotransporter, BSC-1. Am. J. Physiol. 271: F619-F628, 1996. 147. Mandon, B., C.-L. Chou, S. Nielsen, and M.A. Knepper. Syntaxin-4 is localized to the apical plasma membrane of rat renal collecting duct cells: Possible role in aquaporin-2 trafficking. J. Clin. Invest. 98: 906-913, 1996. 148. Ginns, S.M., M.A. Knepper, C.A. Ecelbarger, J. Terris, X. He, R.A. Coleman, J.B. Wade. Immunolocalization of the secretory isoform of Na-K-Cl cotransporter in rat renal intercalated cells. J. Am. Soc. Nephrol. 7: 2533-2542, 1996. 149. Apostol, E, C.A. Ecelbarger, J. Terris, A.D. Bradford, P. Andrews, and M.A. Knepper. Reduced renal medullary water channel expression in puromycin aminonucleoside-induced nephrotic syndrome. J. Am. Soc. Nephrol. 8: 15-24, 1997. 150. Knepper, M.A. Molecular physiology of urinary concentrating mechanism: Regulation of aquaporin water channels by vasopressin. Am. J. Physiol. 272:F3-F12, 1997. 151. Nolan, P.J., M.A. Knepper, and R.K. Packer. Inhibition of IMCD 11ß-hydroxysteroid dehydrogenase type 2 by low pH and acute acid loading. J. Am. Soc. Nephrol. 8: 530-534, 1997. 152. Kishore, B.K., J. Terris, P. Fernández-Llama, and M.A. Knepper. Ultramicro-determination of vasopressin-regulated urea transporter protein in microdissected renal tubules. Am. J. Physiol. 272: F531-F537, 1997. 153. Pallone, T.L., B.K. Kishore, S. Nielsen, P. Agre, and M.A. Knepper. Evidence that aquaporin-1 mediates NaCl-induced water flux across descending vasa recta. Am. J. Physiol. 272: F587-F596, 1997. 154. Ecelbarger, C.A., S. Nielsen, B.R. Olson, T. Murase, E.A. Baker, M.A. Knepper, and J.G. Verbalis. Role of renal aquaporins in escape from vasopressin-induced antidiuresis in rat. J. Clin. Invest. 99: 1852-1863, 1997. 155. Shayakul, C., M.A. Knepper, C.P. Smith, S.R. DiGiovanni, and M.A. Hediger. Segmental localization of urea transporter mRNAs in rat kidney. Am. J. Physiol. 272: F654-F660, 1997. 156. Nielsen, S., J. Terris, D. Andersen, C. Ecelbarger, J. Frokiaer, D. Marples, M.A. Knepper, and J.S. Petersen. Congestive heart failure in rats is associated with increased expression and targeting of aquaporin-2 water channel in collecting duct. Proc. Natl. Acad. Sci. USA 94: 5450-5455, 1997. 157. Knepper, M.A., J.G. Verbalis, and S. Nielsen. Role of aquaporins in water balance disorders. Curr. Opin. Nephrol. Hypertension 6: 367-371, 1997. 158. Knepper, M.A., and T. Inoue. Regulation of aquaporin-2 water channel trafficking by vasopressin. Curr. Opin. Cell Biol. 9: 560-564, 1997. 159. Frokiaer, J., B.M. Christensen, D. Marples, J.C. Djurhuus, U.B. Jensen, M.A. Knepper, and S. Nielsen. Downregulation of aquaporin-2 parallels changes in renal water excretion in unilateral ureteral

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obstruction. Am. J. Physiol. 273: F213-F222, 1997. 160. Nolan, P.J., M.A. Knepper, and R.K. Packer. Role of adrenal steroids in stimulating ammonium excretion during acute metabolic acidosis. Contrib. Nephrol. 121: 55-61, 1997. 161. Mandon, B., S. Nielsen, B.K. Kishore, and M.A. Knepper. Expression of syntaxins in rat kidney. Am. J. Physiol. 273: F718-F730, 1997. 162. Marples, D., T.A. Schroer, N. Aherns, A. Taylor, M.A. Knepper, and S. Nielsen. Dynein and dynactin colocalize with AQP2 water channels in intracellular vesicles from kidney collecting duct. Am. J. Physiol. 274: F384-F394, 1998. 164. Terris, J., C.A. Ecelbarger, J.M. Sands, and M.A. Knepper. Long-term regulation of renal urea transporter protein in rat. J. Am. Soc. Nephrol. 9: 729-736, 1998. 163. Kato, A., M. Naruse, M.A. Knepper, and J.M. Sands. Long-term regulation of inner medullary collecting duct urea transport in rat. J. Am. Soc. Nephrol. 9: 737-745, 1998. 165. Chou, C.-L., T. Ma, B. Yang, M.A. Knepper, A.S. Verkman. Fourfold reduction of water permeability in inner medullary collecting duct of aquaporin-4 knockout mice. Am. J. Physiol. 274: C549-C554, 1998. 166. Chou, C.-L., S.I. Rapko, and M.A. Knepper. Differential effects of carbachol and vasopressin on phosphoinositide signaling in rat inner medullary collecting duct. Am. J. Physiol. 274: F564-F572, 1998. 167. Fernandez-Llama, P, P. Andrews, S. Nielsen, C.A. Ecelbarger, and M.A. Knepper. Impaired aquaporin and urea transporter expression in rats with adriamycin-induced nephrotic syndrome. Kidney International 53: 1244-1253, 1998.

168. Ecelbarger, C.A., C.-L. Chou, A.J. Lee, S.R. DiGiovanni, J.G. Verbalis, and M.A. Knepper. Escape from vasopressin-induced antidiuresis: Role of vasopressin resistance of the collecting duct. Am. J. Physiol. 274: F1161-F1166, 1998. 169. Fernandez-Llama, P, P. Andrews, C.A. Ecelbarger, S. Nielsen, and M.A. Knepper. Concentrating defect in experimental nephrotic syndrome: Altered expression of aquaporins and thick ascending limb Na+ transporters. Kidney International 54: 170-179, 1998. 170. Kishore, B.K., J.B. Wade, K. Schorr, T. Inoue, B. Mandon, and M.A. Knepper. Expression of synaptotagmin VIII in rat kidney. Am. J. Physiol. 275: F131-F142, 1998. 171. Christensen, B.M., D. Marples, U.B. Jensen, J. Frokiaer, D. Sheikh-Hamad, M. Knepper, and S. Nielsen. Acute effects of vasopressin V2-receptor antagonist on kidney AQP2 expression and subcellular distribution. Am. J. Physiol. 275: F285-F297, 1998. 172. Schnermann, J., C.-L. Chou, T. Ma, T. Traynor, M.A. Knepper, and A.S. Verkman. Defective proximal tubular fluid reabsorption in transgenic aquaporin-1 null mice. Proc. Natl. Acad. Sci. USA 95: 9660-9664, 1998.

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173. Knepper, M.A.. Long-term regulation of urinary concentrating capacity. Am. J. Physiol. 275: F332-F333, 1998. 174. Marples, D., B.M. Christensen, J. Frokiaer, M.A. Knepper, and S. Nielsen. Dehydration reverses vasopressin antagonist-induced diuresis and aquaporin-2 downregulation in rats. Am. J. Physiol. 275: F400-F409, 1998. 175. Kwon, T.-H., J. Frokiaer, M.A. Knepper, and S. Nielsen. Reduced AQP1, -2, and -3 levels in kidneys of rats with CRF induced by surgical reduction in renal mass. Am. J. Physiol. 275: F724-F741, 1998. 176. Inoue, T., S. Nielsen, B. Mandon, J. Terris, B.K. Kishore, and M.A. Knepper. SNAP-23 in rat kidney: Co-localization with aquaporin-2 in collecting duct vesicles. Am. J. Physiol. 275: F752-F760, 1998. 177. Kim, G.-H., S. Masilamani, R. Turner, C. Mitchell, J.B. Wade, and M.A. Knepper. The thiazide-sensitive Na-Cl cotransporter is an aldosterone-induced protein. Proc. Natl. Acad. Sci. USA 95: 14552-14557, 1998. 178. Earm, J-H, B. M. Christensen, J. Frokiaer, D. Marples, J.S. Han, M.A. Knepper, S. Nielsen. Decreased aquaporin-2 expression and apical plasma membrane delivery in kidney collecting ducts of polyuric hypercalcemic rats. J. Am. Soc. Nephrol. 9: 2181-2193, 1998. 179. Frokiaer, J., D. Marples, M.A. Knepper, and S. Nielsen. Pathophysiology of aquaporin-2 in water balance disorders. Am. J. Med. Sci. 316: 291-299, 1998. 180. Nielsen, S., A.B. Maunsbach, C.A. Ecelbarger, and M.A. Knepper. Ultrastructural localization of Na-K-2Cl cotransporter in thick ascending limb and macula densa of rat kidney. Am. J. Physiol. 275: F885-F893, 1998. 181. Verbalis, J.G., T. Murase, C.A. Ecelbarger, S. Nielsen, and M.A. Knepper. Studies of renal aquaporin-2 expression during renal escape from vasopressin-induced antidiuresis. Adv. Exp. Med. Biol. 449: 395-406, 1998. 182. Nielsen, S., J. Frokiaer, and M.A. Knepper. Renal aquaporins: key roles in water balance and water balance disorders. Curr. Op. Nephrol. and Hypertension 7: 509-516, 1998. 183. Marples, D., J. Frokiaer, M.A. Knepper, and S. Nielsen. Disordered water channel expression and distribution in acquired nephrogenic diabetes insipidus. Proc. Assoc. Am. Physicians 110: 401-406, 1998. 184. Kim, G.-H., C.A. Ecelbarger, C. Mitchell, R.K. Packer, J.B. Wade, and M.A. Knepper. Vasopressin increases Na-K-2Cl cotransporter expression in thick ascending limb of Henle=s loop. Am. J. Physiol. 276: F96-F103, 1999. 185. Frokiaer, J., D Marples, H. Valtin, J.F. Morris, M.A. Knepper, and S. Nielsen. Low aquaporin-2 levels in polyuric DI +/+ severe mice with constitutively high cAMP-phosphodiesterase activity. Am. J. Physiol. 276: F179-F190, 1999.

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186. Chou, C.L., M.A. Knepper, A. N. van Hoek, D. Brown, T. Ma, and A.S. Verkman. Reduced water permeability and altered ultrastructure in thin descending limb of Henle in aquaporin-1 null mice. J. Clin. Invest. 103: 491-496, 1999. 187. Knepper, M.A., G.-H. Kim, P. Fernández-Llama, and C.A. Ecelbarger. Regulation of thick ascending limb transport by vasopressin. J. Am. Soc. Nephrol. 10: 628-634, 1999. 188. Kim, J., W.-Y. Kim, K- H. Han, M. A. Knepper, S. Nielsen, and K. M. Madsen. Developmental expression of aquaporin 1 in the rat renal vasculature. Am. J. Physiol. 276: F498-F509, 1999. 189. Inoue, T., J. Terris, C.A. Ecelbarger, C.-L. Chou, S. Nielsen, and M.A. Knepper. Vasopressin regulates apical targeting of aquaporin-2 but not of UT1 urea transporter in renal collecting duct. Am. J. Physiol. 276: F559-F566, 1999. 190. Kim, G.-H., C.A. Ecelbarger, M.A. Knepper, and R.K. Packer. Regulation of thick ascending limb ion transporter abundance in response to altered acid-base intake. J. Am. Soc. Nephrol.10: 935-942, 1999. 191. Yasui, M., T.-H. Kwon, M.A. Knepper, S. Nielsen, and P. Agre. Aquaporin-6: an intracellular vesicle water channel protein in renal epithelia. Proc. Natl. Acad. Sci. USA 96:5808-5813, 1999. 192. Fernández-Llama, P, P. Andrews, R. Turner, S. Saggi, J. DiMari, T.-H. Kwon, S. Nielsen, R. Safirstein, and M.A. Knepper. Decreased abundance of collecting duct aquaporins in post-ischemic renal failure in rats. J. Am. Soc. Nephrol. 10:1658-1668, 1999. 193. Fernández-Llama, P, C.A. Ecelbarger, J.A. Ware, P. Andrews, A.J. Lee, R. Turner, S. Nielsen, and M.A. Knepper. Cyclooxygenase inhibitors increase Na-K-2Cl cotransporter abundance in thick ascending limb of Henle=s loop.. Am. J. Physiol. 277:F219-F226, 1999. 194. Fernández-Llama, P, R. Turner, G.F DiBona, and M.A. Knepper. Renal expression of aquaporins in liver cirrhosis induced by chronic common bile duct ligation in rats. J. Am. Soc. Nephrol. 10:1950-1957, 1999. 195. Ecelbarger, C.A., Yu, S., Lee, A.J., Weinstein, L.S., and M.A. Knepper. Decreasaed Na-K-2Cl cotransporter expression in mice with heterozygous disruption of the gene for heterotrimeric G-protein Gsα. Am. J. Physiol. 277:F235-F244, 1999. 196. Murase, T, C.A. Ecelbarger, E.A. Baker, Y. Tian, M.A. Knepper, J.G. Verbalis.. Kidney aquaporin-2 expression during escape from antidiuresis is not related to plasma or tissue osmolality. J. Am. Soc. Nephrol. 10:2067-75, 1999. 197. Kwon, T.H., J. Frokiaer, P. Fernández-Llama, A.B. Maunsbach, M.A. Knepper, and S. Nielsen. Altered expression of Na transporters NHE-3, NaPi-II, Na-K-ATPase, BSC- 1, and TSC in CRF rat. Am. J. Physiol. 277:F257-70, 1999. 198. Kwon, T.H. J. Frøkiaer, P. Fernández-Llama, M. A. Knepper, and S. Nielsen. Reduced abundance of aquaporins in rats with bilateral ischemia-induced acute renal failure: prevention by α-MSH. Am. J. Physiol. 277: F413-F427, 1999.

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199. Masilamani, S. , M.A Knepper,, and M.B. Burg,. Urinary Concentration and Dilution (Chapter 14). In: The Kidney, Brenner, B.M., ed. Philadelphia, Saunders, 1999, pp. 595-635. 200. Masilamani, S., G.-H. Kim, C. Mitchell, J.B. Wade, and M.A. Knepper. Aldosterone-mediated regulation of ENaC α, β and γ subunit proteins in rat kidney. J. Clin. Invest.104: R19-R23, 1999. 201. Wade, J.B., A.J. Lee, J. Liu, C.A. Ecelbarger, C. Mitchell, A.D. Bradford, J. Terris, G.-H. Kim, and M.A. Knepper. UT-A2: A 55 kDa urea transporter in thin descending limb of Henle’s loop whose abundance is regulated by vasopressin. Am. J. Physiol. Renal Physiol. 278: F52-F62, 2000. 202. Kishore, B.K., S.M. Ginns, C.M. Krane, S. Nielsen, and M.A. Knepper. Cellular localization of P2Y2 purinoceptor in rat renal medulla and lung. Am. J. Physiol. Renal Physiol. 278: F43-F51, 2000. 203. Knepper, M.A., H. Valtin, and J. M. Sands. Renal actions of vasopressin (Chapter 13). In: Handbook of Physiology: The Endocrine System., Fray, J.C.S.and H.M. Goodman, eds. New York, Oxford University Press, 2000, pp. 496-529. 204. Agre, P., S. Nielsen and M.A. Knepper. Aquaporin water channels in mammalian kidney (Chapter 15). In: The Kidney: Physiology and Pathophysiology, 3rd Edition. Seldin, D.W., and G. Giebisch, eds., 2000, pp. 363-377. 205. Loffing, J., D. Loffing-Cueni, A. Macher, S.C. Hebert, B. Olson, M. A. Knepper, B.C. Rossier, and B. Kaissling. Localization of epithelial sodium channel and aquaporin-2 in rabbit kidney cortex. Am. J. Physiol. Renal Physiol. 278: F530-F539, 2000. 206. Nielsen, S., T.-H. Kwon, J. Frokiaer, and M.A. Knepper. Key roles of renal aquaporins in water balance and water-balance disorders. News in Physiol. Sci. 15: 136-143, 2000. 207. Ecelbarger, C.A., G.-H. Kim, J. Terris, S. Masilamani, C. Mitchell, I. Reyes, J.G. Verbalis, and M.A. Knepper. Vasopressin-mediated regulation of epithelial sodium channel (ENaC) abundance in rat kidney. Am. J. Physiol. Renal Physiol. 279: F46-F53, 2000. 208. Fernández-Llama, P, W. Jimenez, M. Bosch-Marcé, V. Arroyo, S. Nielsen, and M.A. Knepper. Dysregulation of renal aquaporins and Na-Cl cotransporter in CCl4-induced cirrhosis. Kidney International 58: 216-228, 2000. 209. Kwon, T.H., J. Frokiaer, J.S. Han, M.A. Knepper, and S. Nielsen. Decreased abundance of major Na+ transporters in kidneys of rats with ischemia-induced renal failure. Am. J. Physiol. Renal Physiol. 278: 278: F925-F939, 2000. 210. Promeneur, D., T.-H. Kwon, J. Frøkiær, M. A. Knepper, and S. Nielsen. Vasopressin V2-receptor-dependent regulation of AQP2 expression in Brattleboro rats. Am. J. Physiol. Renal Physiol. 279: F370-F382, 2000. 211. Kim, G.-H., S.W. Martin, P. Fernández-Llama, S. Masilamani, R.K. Packer, and M.A. Knepper. Long-term regulation of renal Na-dependent cotransporters and ENaC: response to altered acid-base intake Am. J. Physiol. Renal Physiol. 279: F459-F467, 2000. 212. Kwon, T.-H., U. H. Laursen, D. Marples, A. B. Maunsbach, M. A. Knepper, J. Frokiaer, and S.

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Nielsen. Altered expression of renal AQPs and Na+ transporters in rats with lithium-induced NDI. Am. J. Physiol. Renal Physiol. 279: F552-F564, 2000. 213. Ho, H.T.B., S.K. Chung, J.W.S. Law, B.C.B. Ko, S.C.F. Tam, H.L. Brooks, M.A. Knepper, and S.S.M Chung. Aldose reductase-deficient mice develop nephrogenic diabetes insipidus. Mol. Cell. Biol. 20:5840-5846, 2000. 214. Stokes, J.B. and M.A. Knepper. Understanding how aldosterone increases sodium transport. Am. J. Kidney Diseases. 36: 866-870, 2000. 215. McKee, J. A., S. Kumar, C.A. Ecelbarger, P. Fernández-Llama, J. Terris. and M.A. Knepper. Detection of Na+ transporter proteins in urine. J. Am. Soc. Nephrol. 11: 2128-2132, 2000. 216. Promeneur, D., T.-H. Kwon, M. Yasui, G.-H. Kim, J. Frokiaer, M.A. Knepper, P. Agre, and S. Nielsen. Regulation of AQP6 mRNA and protein expression in intercalated cells of rat kidney in response to altered acid/alkali load or water load. Am. J. Physiol. Renal Physiol. 279: F1014-F1026, 2000. 217. Chou, C.-L., K.-P. Yip, L. Michea, K. Kador, J. Ferraris, J.B. Wade, and M.A. Knepper. Regulation of aquaporin-2 trafficking by vasopressin in renal collecting duct: Roles of ryanodine-sensitive Ca2+ stores and calmodulin. J. Biol. Chem. 275: 36839-36846, 2000 218. Han, J.S., U.S. Jeon, K.W. Joo, H.J. Chin, W. Huh, J.S. Lee, G.-H. Kim, J.-H. Earm, S. Nielsen, and M.A. Knepper. Oxytocin: One of the factors for regulating AQP2 localization and urinary AQP2 excretion. In: Molecular Biology and Physiology of Water and Solute Transport, Hohman, S., and S. Nielsen, eds. New York, Kluwer Academic/Plenum, 2000, pp. 97-105. 219. Agre P., S. Nielsen S, and M.A. Knepper. Aquaporin water channels in mammalian kidney (Chapter 15). In: The Kidney, Physiology and Pathophysiology, 3rd ed., Seldin, D.W. and G. Giebisch, eds. Lippincott, Williams, and Wilkins, Philadelphia, 2000, pp. 363-377. 220. Ecelbarger, C.A., G.-H. Kim, M. A Knepper, J. Liu, M. Tate, P. A Welling, and J. B. Wade. Regulation of potassium channel Kir 1.1 (ROMK) abundance in the thick ascending limb of Henle's loop. J. Am. Soc. Nephrol. 12: 10-18, 2001. 221. Frindt, G., S. Masilamani, M.A. Knepper, and L. G. Palmer. Activation of epithelial Na channels during short-term Na deprivation. Am. J. Physiol. Renal Physiol. 280: F112-F118, 2001. 222. Ecelbarger, C. A., M. A. Knepper, and J. G. Verbalis. Increased abundance of distal sodium transporters in rat kidney during vasopressin escape. J. Am. Soc. Nephrology.12: 207-217, 2001. 223. Terris, J.M., M.A. Knepper, J.B. Wade. UT-A3: localization and characterization of an additional urea transporter isoform in the IMCD. Am. J. Physiol. Renal Physiol. 280:F325-F332, 2001. 224. Hoffmann, HJ, T. Bjerke, M. Karawajczyk, R. Dahl, M.A. Knepper MA, and S. Nielsen. SNARE proteins are critical for regulated exocytosis of ECP from human eosinophils. Biochem. Biophys. Res. Commun. 280:172-176, 2001. 225. Nejsum, L. N., T.-H. Kwon, D.Marples, A. Flyvbjerg, M.A. Knepper, J. Frøkiær, and S. Nielsen.

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Compensatory increase in AQP2, p-AQP2, and AQP3 expression in rats with diabetes mellitus. Am. J. Physiol. Renal Physiol. 280: F715-F726, 2001. 226. Royaux, I.E., S.M. Wall, L.P. Karniski, L.A. Everett, K. Suzuki, M.A. Knepper, and E.D. Green. Pendrin, encoded by the Pendred Syndrome gene, resides in the apical region of renal intercalated cells and mediates bicarbonate secretion. Proc. Natl. Acad. Sci. USA 98: 4221-4226, 2001. 227. Wang, W., T.-H. Kwon, C. Li, A. Flyvbjerg, M.A. Knepper, J. Frokiaer, and S. Nielsen. Altered expression of renal aquaporins and Na+ transporters in rats treated with the calcium channel blocker. Am. J. Physiol. Reg. 280:R1632-R1641, 2001. 228. Hager, H., T.-H. Kwon, A.K. Vinnikova, S. Masilamani, H. L. Brooks, J. Frokiaer, M.A. Knepper, and S. Nielsen. Immunocytochemical and immunoelectron microscopical localization of α-, β- and γ-ENaC in rat kidney. Am. J. Physiol. Renal Physiol. 280: F1093-F1106, 2001. 229. Knepper, M.A., S. Nielsen, and C.-L. Chou. Physiological roles of aquaporins in the kidney. Current Topics in Membranes 51: 121-153, 2001. 230. Bradford A.D., J.M. Terris, C.A. Ecelbarger, J.D. Klein, J.M. Sands JM, C.L. Chou CL, and M.A. Knepper. 97- and 117-kDa forms of collecting duct urea transporter UT-A1 are due to different states of glycosylation. Am J Physiol Renal Physiol. 281: F133-F143, 2001. 231. Li, C., W. Wang, T.-H. Kwon, L. Isikay, J.G. Wen, D. Marples, J.C. Djurhuus, A. Stockwell, M.A. Knepper, S. Nielsen, and J. Frokiaer. Downregulation of AQP1, -2 and -3 after release of bilateral ureteral obstruction is associated with a long-term urinary concentrating defect. Am. J. Physiol. Renal Physiol. 281: F163-F171, 2001. 232. Wang, X.-Y., S. Masilamani, J. Nielsen, T.-H. Kwon, H. Brooks, S. Nielsen, and M.A. Knepper. The renal thiazide-sensitive Na-Cl cotransporter as mediator of the aldosterone-escape phenomenon. J. Clin. Invest. 108: 215-222, 2001. 233. Knepper, M.A., and H.L. Brooks. Regulation of sodium transporters NHE3, NKCC2 and NCC in the kidney. Curr. Opinion Nephrol. and Hypertension. 10: 655-659, 2001. 234. Bickel, C.A., J. G. Verbalis, M. A. Knepper, and C. A. Ecelbarger. Increased renal Na-K-ATPase, NCC, and β-ENaC abundance in obese Zucker rats. American Journal of Physiol. Renal Physiol. 281: F639-F648, 2001. 235. Kim, Y.-H., J.-H. Earm, T. Ma., A.S. Verkman, M.A. Knepper, K.M. Madsen, and J. Kim. Aquaporin-4 expression in adult and developing mouse and rat kidney. J. Am. Soc. Nephrol. 12: 1795-1804, 2001. 236. Ecelbarger,C.A., G.H. Kim, J.B. Wade, and M.A. Knepper. Regulation of the abundance of renal sodium transporters and channels by vasopressin. Exp Neurol. 171:227-234, 2001. 237. Knepper, M.A., and S. Masilamani. Targeted proteomics in the kidney using ensembles of antibodies. Acta Physiol. Scand. 173: 11-21, 2001. 238. Ecelbarger, C.A., G.H. Kim, J.B. Wade, and M.A. Knepper. Regulation of the abundance of

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renal sodium transporters and channels by vasopressin. Experimental Neurology 171: 227-234, 2001. 239. Nielsen, S., M.A. Knepper, T.-H. Kwon, and J. Frokiaer. Regulation of Water Balance: Urine Concentration and Dilution. In: Diseases of the Kidney and Urinary Tract. R.W. Schrier, ed. Seventh edition. Philadelphia, Lippincott, 2001, pp. 109-134. 240. Brooks, H.L., A.-M. Sorensen, J. Terris, P.J. Schultheis, J.N. Lorenz, G.E. Shull, and M.A. Knepper. Profiling of renal tubule Na+ transporter abundances in NHE3 and NCC null mice using targeted proteomics. J. Physiol. (London) 530: 359-366, 2001. 241. Wang, W., T.H. Kwon, C. Li, J. Frokiaer, M.A. Knepper, S. Nielsen. Reduced expression of Na-K-2Cl cotransporter in medullary thick ascending limbs in vitamin D-induced hypercalcemia in rat. Am J Physiol Renal Physiol. 282: F34-F44, 2002. 242. Nielsen, S., J. Frokiaer, D. Marples, T.H. Kwon, P. Agre, and M. A. Knepper. Aquaporins in the kidney: From molecules to medicine. Physiol. Rev. 82: 205-244, 2002. 243. Brooks, H.L., A.J. Allred, K.T. Beutler, T.M. Coffman, and M.A. Knepper. Targeted proteomic profiling of renal Na+ transporter and channel abundances in AngII type 1a receptor knockout mice. Hypertension 39: 470-473, 2002. 244. Breton, S., T. Inoue, M.A. Knepper, and D. Brown. Antigen-retrieval reveals widespread basolateral expression of syntaxin-3 in renal epithelia. Am. J. Physiol. Renal Physiol. 282: 523-529, 2002. 245. Kim,Y.-H., D.-U. Kim, K.-H. Han, J.-Y. Jung, J.M. Sands, M.A. Knepper, K.M. Madsen, and J. Kim. Expression of urea transporters in the developing rat kidney. Am. J. Physiol. Renal Physiol. 282: 530-540, 2002. 246. Takahashi, N, H.L. Brooks, J.B. Wade, W. Liu, Y. Kondo, S. Ito S, M.A.Knepper, O. Smithies. Posttranscriptional compensation for heterozygous disruption of the kidney-specific NaK2Cl cotransporter gene. J Am Soc Nephrol. 13:604-610, 2002. 247. Wang, X.-Y., K.T. Beutler, J. Nielsen, S. Nielsen, M. A. Knepper, and S. Masilamani. Decreased abundance of collecting duct urea transporters UT-A1 and UT-A3 with ECF volume expansion. Am J Physiol Renal Physiol. 282:577-584, 2002. 248. Itani O.A., S.D. Auerbach, R.F. Husted, K.A. Volk, S. Ageloff, M.A. Knepper, J.B. Stokes, and C.P. Thomas. Glucocorticoid-stimulated lung epithelial Na+ transport is associated with regulated ENaC and sgk1 expression. Am J Physiol Lung Cell Mol Physiol. 282:L631-L641, 2002. 249. Biner H.L, M.P. Arpin-Bott, J. Loffing, X. Wang, M. Knepper, S.C. Hebert, and B. Kaissling. Human cortical distal nephron: distribution of electrolyte and water transport pathways. J Am Soc Nephrol. 2002 13:836-847, 2002. 250. Frank, A.E. , C.S. Wingo, P.M. Andrews, S. Ageloff, M.A. Knepper, and I.D. Weiner. Mechanisms through which ammonia regulates cortical collecting duct net proton secretion. Am J Physiol Renal Physiol 282: F1120-F1128, 2002.

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251. Knepper, M.A. Proteomics and the kidney. J. Am. Soc. Nephrol. 13: 1398-1408, 2002. 252. Bickel, C.A. M.A. Knepper, J.G. Verbalis, and C.A. Ecelbarger. Dysregulation of renal salt and water transport proteins in diabetic Zucker rats. Kidney Int. 61:2099-110, 2002. 253. Manning, J., K. Beutler, M.A. Knepper, and V.M. Vehaskari . Upregulation of renal BSC1 and TSC in prenatally programmed hypertension. Am. J. Physiol. Renal Physiol. 283: 202-206, 2002. 254. Lu M., T. Wang, Q. Yan, X. Yang, K. Dong, M.A. Knepper, W. Wang, G. Giebisch, G.E. Shull, S.C. Hebert SC. Absence of small-conductance K+ channel (SK) activity in apical membranes of thick ascending limb and cortical collecting duct in ROMK (Bartter's) knockout mice. J. Biol. Chem. 277:37881-37887, 2002. 255. Masilamani, S., X.Y. Wang, G.-H. Kim, H.L. Brooks, J. Nielsen, S. Nielsen, K. Nakamura, J.B. Stokes, M.A. Knepper. Time course of changes in renal Na-K-ATPase, NHE3, NKCC2, NCC, and ENaC abundance changes with dietary NaCl restriction. Am. J. Physiol. Renal Physiol. 283: F648-F657, 2002. 256. Nielsen, J. T.-H. Kwon, S. Masilamani, K. Beutler, H. Hager, S. Nielsen, and M.A Knepper. Sodium transporter abundance profiling in kidney: Effect of spironolactone. Am. J. Physiol Renal Physiol. 283: F923-F933, 2002. 257. Wang, W., C. Li, T.-H. Kwon, M. A. Knepper, J. Frokiaer, and S. Nielsen. AQP3, p-AQP2 and AQP2 expression is reduced in polyuric rats with hypercalcemia: prevention by cAMP-PDE inhibitors. Am J Physiol Renal Physiol 283:F1313-F1325, 2002. 258. Elkjaer, M.-L., T.-H. Kwon, W. Wang, J. Nielsen, M. A. Knepper, J. Frokiaer, and S. Nielsen. Altered expression of renal NHE3, TSC, BSC-1, and ENaC subunits in potassium-depleted rats. Am J Physiol Renal Physiol, 283: F1376-F1388, 2002. 259. Kwon, T.-H., J. Nielsen, S. Masilamani, H. Hager, M.A. Knepper, J. Frokiaer, and S. Nielsen. Regulation of collecting duct AQP3 expression: response to mineralocorticoid. Am J Physiol Renal Physiol. 283: F1403-F1421, 2002. 260. Ecelbarger C.A., T. Murase, Y. Tian, S. Nielsen, M.A. Knepper, J.G. Verbalis. Regulation of renal salt and water transporters during vasopressin escape. Prog Brain Res. 139:75-84, 2002. 261. Jeon U.S., K.W. Joo, K.Y. Na, Y.S. Kim, J.S. Lee, J. Kim, G.H. Kim, S. Nielsen, M.A. Knepper, J.S. Han. Oxytocin induces apical and basolateral redistribution of aquaporin-2 in rat kidney. Nephron. 93:E36-E45, 2003 262. Na, K.Y., Y.K. Oh, J. S. Han, K. W. Joo, J. S. Lee, J.-H. Earm, M. A. Knepper, and G.-H. Kim. Upregulation of Na+ transporter abundances in response to chronic thiazide or loop diuretic treatment in rats. Am J Physiol Renal Physiol. 284:F133-F143, 2003. 263. Li, C. W. Wang, T.-H. Kwon, M. A. Knepper, S. Nielsen, and J.Frokiaer. Altered expression of major renal Na transporters in rats with unilateral ureteral obstruction. Am J Physiol Renal Physiol. 284: F155-F166, 2003.

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264. Brooks, H.L. S. Ageloff, T.-H. Kwon, W. Brandt, J. M. Terris, A. Seth, L. Michea, S. Nielsen, R. Fenton, and M.A. Knepper. cDNA array identification of genes regulated in rat renal medulla in response to vasopressin infusion. Am J Physiol Renal Physiol, 284: F218-F228, 2003. 265. Knepper, M.A., G. M. Saidel, V. C. Hascall, and T. Dwyer. Solute concentration in the renal inner medulla: Interstitial hyaluronan as a mechano-osmotic transducer. Am J Physiol Renal Physiol, 284: F433-F446, 2003. 266. Legato, J., M.A. Knepper, R.A. Star, R. Mejia. Database for collecting duct regulatory and transporter proteins. Physiol. Genomics 13:179-181, 2003. 267. Li C., W. Wang, M.A. Knepper, S. Nielsen, J. Frokiaer. Downregulation of renal aquaporins in response to unilateral ureteral obstruction. 284:F1066-F1079, 2003. 268. Kwon, T.-H., J. Nielsen, M.A. Knepper, J. Frøkiær, and S. Nielsen. Regulation of sodium transporters in the thick ascending limb of rat kidney: response to angiotensin II. Am J Physiol Renal Physiol. 285 152-165, 2003. 269. R.A. Fenton, C.-L. Chou, S. Ageloff, W. Brandt, J.B. Stokes, and M.A. Knepper. Increased collecting duct urea transporter expression in Dahl salt-sensitive rats. Am J Physiol Renal Physiol. 285 143-151, 2003. 270. K.T. Beutler, S. Masilamani, S. Turban, J. Nielsen, H.L. Brooks, S. Ageloff, R.A. Fenton, R.K. Packer, and M.A. Knepper. Regulation of ENaC expression in kidney by angiotensin II. Hypertension 41:1143-1150, 2003. 271. M.A. Knepper, G.H. Kim, and S. Masilamani. Renal tubule sodium transporter abundance profiling in rat kidney: response to aldosterone and variations in NaCl intake. Ann N Y Acad Sci. 986:562-569, 2003. 272. Turban, S. X.-Y. Wang, and M.A. Knepper. Regulation of NHE3, NKCC2 and NCC abundance in kidney during aldosterone-escape phenomenon: Role of NO. Am J Physiol Renal Physiol. 285: F843 - 851, 2003. 273. Li C, Wang W, Kwon TH, Knepper MA, Nielsen S, Frokiaer J. Altered expression of major renal Na transporters in rats with bilateral ureteral obstruction and release of obstruction. Am J Physiol Renal Physiol. 285: F889 - F901, 2003. 274. Cadnapaphornchai MA, Summer SN, Falk S, Thurman JM, Knepper MA, Schrier RW. Effect of primary polydipsia on aquaporin and sodium transporter abundance. Am J Physiol Renal Physiol. 285: F965 - F971, 2003. 275. Song J, Knepper MA, Verbalis JG, Ecelbarger CA. Increased renal ENaC subunit and sodium transporter abundances in streptozotocin-induced type I diabetic rats. Am J Physiol Renal Physiol. 285: F1125-F1137, 2003. 276. Nielsen J, Kwon TH, Praetorius J, Kim YH, Frokiaer J, Knepper MA, Nielsen S. Segment specific ENaC downregulation in kidney of rats with lithium-induced NDI. Am J Physiol Renal

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Physiol. 285: F1198-F1211, 2003. 277. Jung JY, Madsen KM, Han KH, Yang CW, Knepper MA, Sands JM, Kim J. Expression of urea transporters in potassium depleted mouse kidney. Am J Physiol Renal Physiol. 285: F1210-F1224, 2003. 278. Hoffert J.D., van Balkom B.W., Chou C.L., Knepper M.A. Application of difference gel electrophoresis (DIGE) to the identification of inner medullary collecting duct proteins. Am J Physiol Renal Physiol. 286:F170-9, 2004. 279. van Balkom B.W., Hoffert J.D., Chou C.L., Knepper M.A. Proteomic analysis of long-term vasopressin action in inner medullary collecting duct of Brattleboro rat. Am J Physiol Renal Physiol. 286: F216-F224, 2004. 280. Grinevich, V., Knepper, M.A., Verbalis, J., Reyes, I., Aguilera, G. Acute endotoxemia in rats induces down-regulation of V2 vasopressin receptors and aquaporin-2 content in the kidney medulla. Kidney Int. 65:54-62, 2004. 281. Wang W, Li C, Kwon TH, Miller RT, Knepper MA, Frokiaer J, Nielsen S. Reduced expression of renal Na+ transporters in rats with PTH-induced hypercalcemia. Am J Physiol Renal Physiol. 2004; 286:F534-F545. 282. Vinnikova AK, Alam RI, Malik SA, Ereso GL, Feldman GM, McCarty JM, Knepper MA, Heck GL, DeSimone JA, Lyall V. Na+-H+ exchange activity in taste receptor cells. J Neurophysiol. 2004; 91:1297-1313. 283. Song J, Knepper MA, Hu X, Verbalis JG, Ecelbarger CA. Rosiglitazone activates renal sodium- and water-reabsorptive pathways and lowers blood pressure in normal rats. J Pharmacol Exp Ther. 2004; 308:426-433. 284. Knepper MA, Nielsen S. Peter Agre, 2003 Nobel Prize winner in chemistry. J Am Soc Nephrol. 2004; 15:1093-1095. Comment. 285. Kim SW, Wang W, Nielsen J, Praetorius J, Kwon TH, Knepper MA, Frokiaer J, Nielsen S. Increased expression and apical targeting of renal ENaC subunits in puromycin aminonucleoside-induced nephrotic syndrome in rats. Am J Physiol Renal Physiol. 2004; 286:F922-F935. 286. Hoagland KM, Flasch AK, Dahly-Vernon AJ, Dos Santos EA, Knepper MA, Roman RJ. Elevated BSC-1 and ROMK expression in Dahl salt-sensitive rat kidneys. Hypertension. 2004; 43:860-865. 287. Shi Y, Pedersen M, Li C, Wen JG, Thomsen K, Stodkilde-Jorgensen H, Jorgensen TM, Knepper MA, Nielsen S, Djurhuus JC, Frokiaer J. Early release of neonatal ureteral obstruction preserves renal function. Am J Physiol Renal Physiol. 2004; 286:F1087-99. 288. Lim SW, Li C, Sun BK, Han KH, Kim WY, Oh YW, Lee JU, Kador PF, Knepper MA, Sands JM, Kim J, Yang CW. Long-term treatment with cyclosporine decreases aquaporins and urea transporters in the rat kidney. Am J Physiol Renal Physiol. 2004; 287:F139-51.

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289. Li C, Klein JD, Wang W, Knepper MA, Nielsen S, Sands JM, Frokiaer J. Altered expression of urea transporters in response to ureteral obstruction. Am J Physiol Renal Physiol. 2004; 286:F1154-62. 290. Shi Y, Li C, Thomsen K, Jorgensen TM, Knepper MA, Nielsen S, Djurhuus JC, Frokiaer J. Neonatal ureteral obstruction alters expression of renal sodium transporters and aquaporin water channels. Kidney Int. 2004; 66:203-215. 291. Fenton RA, Chou CL, Stewart GS, Smith CP, Knepper MA. Urinary concentrating defect in mice with selective deletion of phloretin-sensitive urea transporters in the renal collecting duct. Proc Natl Acad Sci U S A. 2004; 101:7469-74. 292. M.A Knepper, M.A. and G. Gamba,. Urinary Concentration and Dilution (Chapter 14). In: The Kidney, Brenner, B.M., ed. Philadelphia, Saunders, 2004, pp. 599-636. 293. Song J, Hu X, Shi M, Knepper MA, Ecelbarger CA. Effects of dietary fat, NaCl, and fructose on renal sodium and water transporter abundances and systemic blood pressure. Am J Physiol Renal Physiol. 2004; 287: F1204-F1212. 294. Singh BB, Lockwich TP, Bandyopadhyay BC, Liu X, Bollimuntha S, Brazer SC, Combs C, Das S, Leenders AG, Sheng ZH, Knepper MA, Ambudkar SV, Ambudkar IS. VAMP2-dependent exocytosis regulates plasma membrane insertion of TRPC3 channels and contributes to agonist-stimulated Ca2+ influx. Mol Cell. 2004;15:635-646. 295. Pisitkun T, Shen RF, Knepper MA. Identification and proteomic profiling of exosomes in human urine. Proc Natl Acad Sci U S A. 2004; 101:13368-13373. 296. Chou CL, Christensen BM, Frische S, Vorum H, Desai RA, Hoffert JD, De Lanerolle P, Nielsen S, Knepper MA. Nonmuscle myosin II and myosin light chain kinase are downstream targets for vasopressin signaling in the renal collecting duct. J Biol Chem. 2004 19; 279: 49026-49035. 297. Knepper MA, Agre P. Structural biology. The atomic architecture of a gas channel. Science. 2004;305:1573-1574. Comment. 298. Kim GH, Lee JW, Oh YK, Chang HR, Joo KW, Na KY, Earm JH, Knepper MA, Han JS. Antidiuretic effect of hydrochlorothiazide in lithium-induced nephrogenic diabetes insipidus is associated with upregulation of aquaporin-2, Na-Cl co-transporter, and epithelial sodium channel. J Am Soc Nephrol. 2004; 15:2836-2843. 299. Kwon TH, Nielsen J, Knepper MA, Frokiaer J, Nielsen S. Angiotensin II AT1 receptor blockade decreases vasopressin-induced water reabsorption and AQP2 levels in NaCl-restricted rats. Am J Physiol Renal Physiol. 2005; 288:F673-F684. 300. Hoffert JD, Chou CL, Fenton RA, Knepper MA. Calmodulin is required for vasopressin-stimulated increase in cyclic AMP production in inner medullary collecting duct. J Biol Chem. 2005; 280:13624-13630. 301. Fernández-Llama, P., Ageloff S, Fernandez-Varo G, Ros J, Wang X, Garra N,

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Esteva-Font C, Ballarin J, Barcelo P, Arroyo V, Stokes JB, Knepper MA, Jimenez W. Sodium retention in cirrhotic rats is associated with increased renal abundances of sodium transporter proteins. Kidney Int. 2005; 67: 622-630. 302. Fenton RA, Flynn A, Shodeinde A, Smith CP, Schnermann J, Knepper MA. Renal phenotype of UT-A urea transporter knockout mice. J Am Soc Nephrol. 2005; 16: 1583-1592. 303. Morris RG, Uchida S, Brooks H, Knepper MA, Chou CL. Altered expression profile of transporters in the IMCD of aquaporin 1 knockout mice.. Am J Physiol Renal Physiol. 2005; 289: F194-F199. 304. Norregaard R, Jensen BL, Li C, Wang W, Knepper MA, Nielsen S, Frokiaer J. COX-2 inhibition prevents downregulation of key renal water and sodium transport proteins in response to bilateral ureteral obstruction. Am J Physiol Renal Physiol. 2005; 289: F322-F333. 305. Barile M, Pisitkun T, Yu MJ, Chou CL, Verbalis MJ, Shen RF, Knepper MA. Large-scale protein identification in intracellular aquaporin-2 vesicles from renal inner medullary collecting duct. Mol Cell Proteomics. 2005; 4: 1095-1106. 306. Kim SW, Wang W, Kwon TH, Knepper MA, Frokiaer J, Nielsen S. Increased expression of ENaC subunits and increased apical targeting of AQP2 in the kidneys of spontaneously hypertensive rats. Am J Physiol Renal Physiol. 2005; 289: F957-F968. 307. Hoorn EJ, Pisitkun T, Zietse R, Gross P, Frokiaer J, Wang NS, Gonzales PA, Star RA, Knepper MA. Prospects for urinary proteomics: exosomes as a source of urinary biomarkers. Nephrology. 2005; 10: 283-290. Review. 308. Hoorn EJ, Hoffert JD, Knepper MA. Combined proteomics and pathways analysis of collecting duct reveals a protein regulatory network activated in vasopressin escape. J Am Soc Nephrol. 2005; 16: 2852-2863. 309. Frokiaer J, Nielsen S, Knepper MA. Molecular physiology of renal aquaporins and sodium transporters: exciting approaches to understand regulation of renal water handling. J Am Soc Nephrol. 2005; 16: 2827-2829. Comment. 310. Kim SW, Schou UK, Peters CD, de Seigneux S, Kwon TH, Knepper MA, Jonassen TE, Frokiaer J, Nielsen S. Increased apical targeting of renal epithelial sodium channel subunits and decreased expression of type 2 11β-hydroxysteroid dehydrogenase in rats with CCl4-induced decompensated liver cirrhosis. J Am Soc Nephrol. 2005 16: 3196-3210. 311. Kim SW, Wang W, Sassen MC, Choi KC, Han JS, Knepper MA, Jonassen TE, Frokiaer J, Nielsen S. Biphasic changes of epithelial sodium channel abundance and trafficking in common bile duct ligation-induced liver cirrhosis. Kidney Int. 2006; 69: 89-98. 312. Fenton RA, Shodeinde A, Knepper MA. UT-A urea transporter promoter, UT-A targets principal cells of the renal inner medullary collecting duct. Am J Physiol Renal Physiol. 2006; 290: F188-F195. 313. Li C, Shi Y, Wang W, Sardeli C, Kwon TH, Thomsen K, Jonassen T, Djurhuus JC, Knepper

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MA, Nielsen S, Frokiaer J. αMSH prevents impairment in renal function and dysregulation of AQPs and Na-K-ATPase in rats with bilateral ureteral obstruction. Am J Physiol Renal Physiol. 2006; 290:F384-F396. 314. Wall SM, Knepper MA, Hassell KA, Fischer MP, Shodeinde A, Shin W, Pham TD, Meyer JW, Lorenz JN, Beierwaltes WH, Dietz JR, Shull GE, Kim YH. Hypotension in NKCC1 null mice: role of the kidneys. Am J Physiol Renal Physiol. 2006; 290:F409-F416. 315. Nielsen J, Kwon TH, Praetorius J, Frokiaer J, Knepper MA, Nielsen S. Aldosterone increases urine production and decreases apical AQP2 expression in rats with diabetes insipidus. Am J Physiol Renal Physiol. 2006; 290:F438-F449. 316. Wang W, Li C, Nejsum LN, Li H, Kim SW, Kwon TH, Jonassen TE, Knepper MA, Thomsen K, Frokiaer J, Nielsen S. Biphasic effects of ANP infusion in conscious, euvolumic rats: roles of AQP2 and ENaC trafficking. Am J Physiol Renal Physiol. 2006; 290:F530-F541. 317. Pruitt ME, Knepper MA, Graves B, Schmidt-Nielsen B. Effect of peristaltic contractions of the renal pelvic wall on solute concentrations of the renal inner medulla in the hamster. Am J Physiol Renal Physiol. 2006; 290:F892-F896. 318. Nielsen J, Kwon TH, Frokiaer J, Knepper MA, Nielsen S. Lithium-induced NDI in rats is associated with loss of αENaC regulation by aldosterone in CCD. Am J Physiol Renal Physiol. 2006; 290:F1222-F1233. 319. Lim SW, Han KH, Jung JY, Kim WY, Yang CW, Sands JM, Knepper MA, Madsen KM, Kim J. Ultrastructural localization of UT-A and UT-B in rat kidneys with different hydration status. Am J Physiol Regul Integr Comp Physiol. 2006; 290:R479-R492. 320. Zhou H, Yuen PS, Pisitkun T, Gonzales PA, Yasuda H, Dear JW, Gross P, Knepper MA, Star RA. Collection, storage, preservation, and normalization of human urinary exosomes for biomarker discovery. Kidney Int. 2006; 69:1471-1476. 321. Kim SW, de Seigneux S, Sassen MC, Lee J, Kim J, Knepper MA, Frokiaer J, Nielsen S. Increased apical targeting of renal ENaC subunits and decreased expression of 11HSD2 in HgCl2-induced nephrotic syndrome in rats. Am J Physiol Renal Physiol. 2006; 290:F674-F687. 322. Morris RG, Hoorn EJ, Knepper MA. Hypokalemia in a mouse model of Gitelman's syndrome. Am J Physiol Renal Physiol. 2006; 290:F1416-F1420. 323. Pisitkun T, Bieniek J, Tchapyjnikov D, Wang G, Wu WW, Shen RF, Knepper MA. High-throughput identification of IMCD proteins using LC-MS/MS. Physiol Genomics. 2006; 25:263-276. 324. Hoffert JD, Pisitkun T, Wang G, Shen RF, Knepper MA. Quantitative phosphoproteomics of vasopressin-sensitive renal cells: Regulation of aquaporin-2 phosphorylation at two sites. Proc Natl Acad Sci U S A. 2006; 103:7159-7164. 325. Fenton RA, Chou CL, Sowersby H, Smith CP, Knepper MA. Gamble's 'Economy of Water' revisited: Studies in urea transporter knockout mice. Am J Physiol Renal Physiol. 2006;

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291:F148-F154. 326. Hoorn EJ, Hoffert JD, Knepper MA. The application of DIGE-based proteomics to renal physiology. Nephron Physiol. 2006;104:61-72. Review. 327. Jensen AM, Li C, Praetorius HA, Norregaard R, Frische S, Knepper MA, Nielsen S, Frokiaer J. Angiotensin II mediates downregulation of aquaporin water channels and key renal sodium transporters in response to urinary tract obstruction. Am J Physiol Renal Physiol. 2006; 291:F1021-F1032. 328. Turban S, Beutler KT, Morris RG, Masilamani S, Fenton RA, Knepper MA, Packer RK. Long-term regulation of proximal tubule acid-base transporter abundance by angiotensin II. Kidney Int. 2006; 70:660-668. 329. Pisitkun T, Johnstone R, Knepper MA. Discovery of urinary biomarkers. Mol Cell Proteomics. 2006; 5:1760-1771. Review. 330. Sassen MC, Kim SW, Kwon TH, Knepper MA, Miller RT, Frokiaer J, Nielsen S. Dysregulation of renal sodium transporters in gentamicin-treated rats. Kidney Int. 2006; 70:1026-1037. 331. Simons BL, Wang G, Shen RF, Knepper MA. In vacuo isotope coded alkylation technique (IVICAT); an N-terminal stable isotopic label for quantitative liquid chromatography/mass spectrometry proteomics. Rapid Commun Mass Spectrom. 2006; 20:2463-2477. 332. Yu MJ, Pisitkun T, Wang G, Shen RF, Knepper MA. LC-MS/MS analysis of apical and basolateral plasma membranes of rat renal collecting duct cells. Mol Cell Proteomics. 2006; 5:2131-2145.

333. Wang G, Wu WW, Pisitkun T, Hoffert JD, Knepper MA, Shen RF. Automated quantification tool for high-throughput proteomics using stable isotope labeling and LC-MSn. Anal Chem. 2006; 78:5752-5761. 334. Zhou H, Pisitkun T, Aponte A, Yuen PS, Hoffert JD, Yasuda H, Hu X, Chawla L, Shen RF, Knepper MA, Star RA. Exosomal Fetuin-A identified by proteomics: a novel urinary biomarker for detecting acute kidney injury. Kidney Int. 2006; 70: 1847-1857. 335. Fenton RA, Smith CP, Knepper MA. Role of collecting duct urea transporters in the kidney--insights from mouse models. J Membr Biol. 2006; 212:119-131. Review. 336. Curthoys NP, Taylor L, Hoffert JD, Knepper MA. Proteomic analysis of the adaptive response of rat renal proximal tubules to metabolic acidosis. Am J Physiol. 2007; 292:F140-147. 337. Nielsen J, Kwon TH, Frokiaer J, Knepper MA, Nielsen S. Maintained ENaC trafficking in aldosterone-infused rats during mineralocorticoid and glucocorticoid receptor blockade. Am J Physiol. 2007; 292:F382-F394. 338. Hoffert JD, Nielsen J, Yu MJ, Pisitkun T, Schleicher SM, Nielsen S, Knepper MA. Dynamics of aquaporin-2 serine-261 phosphorylation in response to short-term vasopressin treatment in

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collecting duct. Am J Physiol. 2007; 292:F691-F700. 339. Topcu SO, Pedersen M, Norregaard R, Wang G, Knepper M, Djurhuus JC, Nielsen S, Jorgensen TM, Frokiaer J. Candesartan prevents long-term impairment of renal function in response to neonatal partial unilateral ureteral obstruction. Am J Physiol. 2007; 292:F736-F748. 340. Norregaard R, Jensen BL, Topcu SO, Diget M, Schweer H, Knepper MA, Nielsen S, Frokiaer J. COX-2 activity transiently contributes to increased water and NaCl excretion in the polyuric phase after release of ureteral obstruction. Am J Physiol. 2007; 292:F1322-F1333. 341. Cheruvanky A, Zhou H, Pisitkun T, Kopp JB, Knepper MA, Yuen PS, Star RA. Rapid isolation of urinary exosomal biomarkers using a nanomembrane ultrafiltration concentrator. Am J Physiol. 2007; 292:F1657-F1661. 342. Fenton RA, Knepper MA. Urea and renal function in the 21st century: insights from knockout mice. J Am Soc Nephrol. 2007; 18:679-688. Review. 343. Na KY, Kim GH, Joo KW, Lee JW, Jang HR, Oh YK, Jeon US, Chae SW, Knepper MA, Han JS. Chronic furosemide or hydrochlorothiazide administration increases H+-ATPase B1 subunit abundance in rat kidney. Am J Physiol. 2007; 292:F1701-F1709. 344. Li C, Wang W, Norregaard R, Knepper MA, Nielsen S, Frokiaer J. Altered expression of epithelial sodium channel (ENaC) in rats with bilateral or unilateral ureteral obstruction. Am J Physiol. 2007; 293:F333-F341. 345. Wu WW, Wang G, Yu MJ, Knepper MA, Shen RF. Identification and quantification of basic and acidic proteins using solution-based two-dimensional protein fractionation and label-free or 18O-labeling mass spectrometry. J Proteome Res. 2007; 6:2447-2459. 346. Esteva-Font C, Ars E, Guillen-Gomez E, Campistol JM, Sanz L, Jimenez W, Knepper MA, Torres F, Torra R, Ballarin JA, Fernandez-Llama P. Cyclosporine-induced hypertension is associated with increased sodium transporter of the loop of Henle (NKCC2). Nephrol Dial Transplant. 2007; 22:2810-2816. 347. Dvorak MM, De Joussineau C, Carter DH, Pisitkun T, Knepper MA, Gamba G, Kemp PJ, Riccardi D. Thiazide diuretics directly induce osteoblast differentiation and mineralized nodule formation by interacting with a sodium chloride co-transporter in bone. J Am Soc Nephrol. 2007; 18:2509-2516. 348. Oh YK, Joo KW, Lee JW, Jeon US, Lim CS, Han JS, Knepper MA, Na KY. Altered renal sodium transporter expression in an animal model of type 2 diabetes mellitus. J Korean Med Sci. 2007; 22:1034-1041. 349. Hoffert JD, Wang G, Pisitkun T, Shen RF, Knepper MA. An automated platform for analysis of phosphoproteomic datasets: application to kidney collecting duct phosphoproteins. J Proteome Res. 2007 6:3501-3508. 350. Pisitkun T, Hoffert JD, Yu MJ, Knepper MA. Tandem mass spectrometry in physiology. Physiology (Bethesda). 2007; 22: 390-400. Review.

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351. Fenton RA, Knepper MA. Mouse models and the urinary concentrating mechanism in the new millennium. Physiol Rev. 2007; 87:1083-112. Review. 352. Knepper MA, Pisitkun T. Exosomes in urine: who would have thought...? Kidney Int. 2007; 72:1043-5. Comment. 353. Hoffert JD, Knepper MA. Taking aim at shotgun phosphoproteomics. Anal Biochem. 2008; 375: 1-10. Review. 354. Picard N, Eladari D, El Moghrabi S, Planes C, Bourgeois S, Houillier P, Wang Q, Burnier M, Deschenes G, Knepper MA, Meneton P, Chambrey R. Defective ENaC processing and function in tissue-kallikrein deficient mice. J Biol Chem. 2008; 283:4602-11. 355. Uawithya P, Pisitkun T, Ruttenberg BE, Knepper MA. Transcriptional profiling of native inner medullary collecting duct cells from rat kidney. Physiol Genomics. 2008; 32:229-253. 356. Kim GH, Choi NW, Jung JY, Song JH, Lee CH, Kang CM, Knepper MA. Treating lithium-induced nephrogenic diabetes insipidus with a COX-2 inhibitor improves polyuria via upregulation of AQP2 and NKCC2. Am J Physiol 2008; 294:F702-9. 357. Knepper MA, Star RA. Vasopressin: friend or foe? Nature Med. 2008; 14:14-16. Comment. 358. Hoorn EJ, Pisitkun T, Yu MJ, Knepper MA. Proteomic approaches for the study of cell signaling in the renal collecting duct. Contrib Nephrol. 2008; 160:172-185. Review. 359. Fenton RA, Moeller HB, Hoffert JD, Yu MJ, Nielsen S, Knepper MA. Acute regulation of aquaporin-2 phosphorylation at Ser-264 by vasopressin. Proc Natl Acad Sci U S A. 2008; 105:3134-3139. 360. Nielsen J, Hoffert JD, Knepper MA, Agre P, Nielsen S, Fenton RA. Proteomic analysis of lithium-induced nephrogenic diabetes insipidus: mechanisms for aquaporin 2 down-regulation and cellular proliferation. Proc Natl Acad Sci U S A. 2008; 105:3634-3639. 361. Gonzales P, Pisitkun T, Knepper MA. Urinary exosomes: is there a future? Nephrol Dial Transplant. 2008; 23:1799-1801. Comment. 362. Chou CL, Yu MJ, Kassai EM, Morris RG, Hoffert JD, Wall SM, Knepper MA. Role of basolateral solute uptake via NKCC1 and of myosin II in vasopressin-induced cell swelling in inner medullary collecting duct. Am J Physiol. 2008; 295: F192-201. 363. Hao JJ, Wang G, Pisitkun T, Patino-Lopez G, Nagashima K, Knepper MA, Shen RF, Shaw S. Enrichment of distinct microfilament-associated and GTP-binding-proteins in membrane/microvilli fractions from lymphoid cells. J Proteome Res. 2008; 7:2911-27. 364. Ruttenberg BE, Pisitkun T, Knepper MA, Hoffert JD. PhosphoScore: An open-source phosphorylation site assignment tool for MSn data. J Proteome Res. 2008; 7:3054-9. 365: Yu MJ, Pisitkun T, Wang G, Aranda JF, Gonzales PA, Tchapyjnikov D, Shen RF,

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Alonso MA, Knepper MA. Large-scale quantitative LC-MS/MS analysis of detergent-resistant membrane proteins from rat renal collecting duct. Am J Physiol Cell Physiol. 2008; 295: C661-78. 366. Hoffert JD, Fenton RA, Moeller HB, Simons B, Tchapyjnikov D, McDill BW, Yu MJ, Pisitkun T, Chen F, Knepper MA. Vasopressin-stimulated increase in phosphorylation at Ser269 potentiates plasma membrane retention of aquaporin-2. J Biol Chem. 2008; 283:24617-27. 367. Jacob VA, Harbaugh CM, Dietz JR, Fenton RA, Kim SM, Castrop H, Schnermann J, Knepper MA, Chou CL, Anderson SA. Magnetic resonance imaging of urea transporter knockout mice shows renal pelvic abnormalities. Kidney Int. 2008; 74: 1202-8. 368. Pisitkun T, Jacob VA, Schleicher SM, Chou CL, Yu MJ, Knepper MA. Akt and ERK1/2 pathways are components of the vasopressin signaling network in rat native IMCD. Am J Physiol Renal Physiol. 2008; 295: F1030-43. 369. Sachs AN, Pisitkun T, Hoffert JD, Yu MJ, Knepper MA. LC-MS/MS analysis of differential centrifugation fractions from native inner medullary collecting duct of rat. Am J Physiol Renal Physiol. 2008; 295:F1799-F1806. 370. Knepper MA. Courier service for ammonia. Nature. 2008; 456:336-337. Comment. 371. Moeller HB, Knepper MA, Fenton RA. Serine 269 phosphorylated aquaporin-2 is targeted to the apical membrane of collecting duct principal cells. Kidney Int. 2009; 75: 295-303. 372. Moeller HB, Macaulay N, Knepper MA, Fenton RA. Role of multiple phosphorylation sites in the carboxyl-terminal tail of aquaporin-2 for water transport: Evidence against channel gating. Am J Physiol Renal Physiol. 2009 Am J Physiol Renal Physiol. 2009; 296:649-57. 373. Gonzales PA, Pisitkun T, Hoffert JD, Tchapyjnikov D, Star RA, Kleta R, Wang NS, Knepper MA. Large-scale proteomics and phosphoproteomics of urinary exosomes. J Am Soc Nephrol. 2009; 2:363-79. 374. Yu MJ, Miller RL, Uawithya P, Rinschen MM, Khositseth S, Braucht DW, Chou CL, Pisitkun T, Nelson RD, Knepper MA. Systems-level analysis of cell-specific AQP2 gene expression in renal collecting duct. Proc Natl Acad Sci U S A. 2009; 106:2441-6. 375. Hoffert JD, Chou CL, Knepper MA. Aquaporin-2 in the '-omics' era. J Biol Chem. 2009;284: 14683-7376. 376. Zwang NA, Hoffert JD, Pisitkun T, Moeller HB, Fenton RA, Knepper MA. Identification of phosphorylation-dependent binding partners of aquaporin-2 using protein mass spectrometry. J Proteome Res. 2009; 8:1540-54. 377. Bockenhauer D, Feather S, Stanescu HC, Bandulik S, Zdebik AA, Reichold M, Tobin J, Lieberer E, Sterner C, Landoure G, Arora R, Sirimanna T, Thompson D, Cross JH, van't Hoff W, Al Masri O, Tullus K, Yeung S, Anikster Y, Klootwijk E, Hubank M, Dillon MJ, Heitzmann D, Arcos-Burgos M, Knepper MA, Dobbie A, Gahl WA, Warth R, Sheridan E, Kleta R. Epilepsy, ataxia, sensorineural deafness, tubulopathy, and KCNJ10 mutations. N Engl J Med. 2009;360:1960-70.

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378. Knepper MA. Common sense approaches to urinary biomarker study design. J Am Soc Nephrol. 2009;20:1175-8. 379. Li JH, Chou CL, Li B, Gavrilova O, Eisner C, Schnermann J, Anderson SA, Deng CX, Knepper MA, Wess J. A selective EP4 PGE2 receptor agonist alleviates disease in a new mouse model of X-linked nephrogenic diabetes insipidus. J Clin Invest.2009: 119:3115-3126. 380. Lütken SC, Kim SW, Jonassen T, Marples D, Knepper MA, Kwon TH, Frøkiaer J, Nielsen S. Changes of renal AQP2, ENaC, and NHE3 in experimentally induced heart failure: response to angiotensin II AT1 receptor blockade. Am J Physiol Renal Physiol. 2009; 297:F1678-88. 381. Knepper MA, Mindell JA. Structural biology: Molecular coin slots for urea. Nature. 2009;462:733-4. 382. Tchapyjnikov D, Li Y, Pisitkun T, Hoffert JD, Yu MJ, Knepper MA. Proteomic profiling of nuclei from native renal inner medullary collecting duct cells using LC-MS/MS. Physiol Genomics. 2010; 40: 167-83. 383. Fernández-Llama P, Khositseth S, Gonzales PA, Star RA, Pisitkun T, Knepper MA. Tamm-Horsfall protein and urinary exosome isolation. Kidney Int. 2010; 77: 736-42. 384. Xie L, Hoffert JD, Chou CL, Yu MJ, Pisitkun T, Knepper MA, Fenton RA. Quantitative analysis of aquaporin-2 phosphorylation. Am J Physiol Renal Physiol. 2010; 298: F1018-23. 385. Bansal AD, Hoffert JD, Pisitkun T, Hwang S, Chou CL, Boja ES, Wang G, Knepper MA. Phosphoproteomic profiling reveals vasopressin-regulated phosphorylation sites in collecting duct. J Am Soc Nephrol. 2010; 21:303-15. 386. Da Silva N, Pisitkun T, Belleannée C, Miller LR, Nelson RD, Knepper MA, Brown D, Breton S. Proteomic analysis of V-ATPase-rich cells harvested from the kidney and epididymis by fluorescence activated cell sorting. Am J Physiol Cell Physiol. 2010; 298: C1326-42. 387. Rinschen MM, Yu MJ, Wang G, Boja ES, Hoffert JD, Pisitkun T, Knepper MA. Quantitative phosphoproteomic analysis reveals vasopressin V2-receptor-dependent signaling pathways in renal collecting duct cells. Proc Natl Acad Sci U S A. 2010; 107:3882-7. 388. Gonzales PA, Zhou H, Pisitkun T, Wang NS, Star RA, Knepper MA, Yuen PS. Isolation and purification of exosomes in urine. Methods Mol Biol. 2010; 641: 89-99. 389. Hwang S, Gunaratne R, Rinschen MM, Yu MJ, Pisitkun T, Hoffert JD, Fenton RA, Knepper MA, Chou CL. Vasopressin increases phosphorylation of Ser84 and Ser486 in Slc14a2 collecting duct urea transporters. Am J Physiol Renal Physiol. 2010; 299: F559-67. 390. Gunaratne R, Braucht DW, Rinschen MM, Chou CL, Hoffert JD, Pisitkun T, Knepper MA. Quantitative phosphoproteomic analysis reveals cAMP/vasopressin-dependent signaling pathways in native renal thick ascending limb cells. Proc Natl Acad Sci U S A. 2010; 107: 15653-8. 391. Khositseth S, Pisitkun T, Slentz DH, Wang G, Hoffert JD, Knepper MA, Yu MJ. Quantitative protein and mRNA profiling shows selective post-transcriptional control of protein expression by

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vasopressin in kidney cells. Mol Cell Proteomics. 2011: M110.004036. 392. Feric M, Zhao B, Hoffert JD, Pisitkun T, Knepper MA. Large-scale phosphoproteomic analysis of membrane proteins in renal proximal and distal tubule. Am J Physiol Cell Physiol. 2011; 300: C755-70. 393. Stødkilde L, Nørregaard R, Fenton RA, Wang G, Knepper MA, Frøkiær J. Bilateral ureteral obstruction induces early downregulation and redistribution of AQP2 and phosphorylated AQP2. Am J Physiol Renal Physiol. 2011; 301: F226-F235. 394. Hoffert JD, Pisitkun T, Knepper MA. Phosphoproteomics of vasopressin signaling in the kidney. Expert Rev Proteomics. 2011; 8: 157-63. 395. van Balkom BW, Pisitkun T, Verhaar MC, Knepper MA. Exosomes and the kidney: prospects for diagnosis and therapy of renal diseases. Kidney Int. 2011; 80: 1138-45. 396. Zhao B, Knepper MA, Chou CL, Pisitkun T. Large-scale phosphotyrosine proteomic profiling of rat renal collecting duct epithelium reveals predominance of proteins involved in cell polarity determination. Am J Physiol Cell Physiol. 2012; 302: C27-45. 397. Pisitkun T, Hoffert JD, Saeed F, Knepper MA. NHLBI-AbDesigner: An online tool for design of peptide-directed antibodies. Am J Physiol Cell Physiol. 2012; 302: C154-64. 398. Huling JC, Pisitkun T, Song JH, Yu MJ, Hoffert JD, Knepper MA. Gene expression databases for kidney epithelial cells. Am J Physiol Renal Physiol. 2012; 302: F401-7. 399. Hoffert JD, Pisitkun T, Saeed F, Song JH, Chou CL, Knepper MA. Dynamics of the G protein-coupled vasopressin V2 receptor signaling network revealed by quantitative phosphoproteomics. Mol Cell Proteomics. 2012; 11: M111.014613. 400. Schenk LK, Bolger SJ, Luginbuhl K, Gonzales PA, Rinschen MM, Yu MJ, Hoffert JD, Pisitkun T, Knepper MA. Quantitative proteomics identifies vasopressin-responsive nuclear proteins in collecting duct cells. J Am Soc Nephrol. 2012; 23: 1008-18. 401. Radin MJ, Yu MJ, Stoedkilde L, Miller RL, Hoffert JD, Frokiaer J, Pisitkun T, Knepper MA. Aquaporin-2 regulation in health and disease. Vet Clin Pathol. 2012; 41: 455-70. Review. 402. Zhao B, Pisitkun T, Hoffert JD, Knepper MA, Saeed F. CPhos: a program to calculate and visualize evolutionarily conserved functional phosphorylation sites. Proteomics. 2012;12:3299-303. 403. Bolger SJ, Hurtado PA, Hoffert JD, Saeed F, Pisitkun T, Knepper MA. Quantitative phosphoproteomics in nuclei of vasopressin-sensitive renal collecting duct cells. Am J Physiol Cell Physiol. 2012; 303: C1006-20. 404. Knepper MA. Systems biology in physiology: the vasopressin signaling network in kidney. Am J Physiol Cell Physiol. 2012; 303: C1115-24. Review. 405. Douglass J, Gunaratne R, Bradford D, Saeed F, Hoffert JD, Steinbach PJ, Knepper MA, Pisitkun T. Identifying protein kinase target preferences using mass spectrometry. Am J Physiol Cell

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Physiol. 2012; 303: C715-27. 406. Vilbig RL, Sarkar A, Zischkau J, Knepper MA, Pisitkun T. An online tool for calculation of free-energy balance for the renal inner medulla. Am J Physiol Renal Physiol. 2012; 303: F366-72. 407. Pisitkun T, Gandolfo MT, Das S, Knepper MA, Bagnasco SM. Application of systems biology principles to protein biomarker discovery: urinary exosomal proteome in renal transplantation. Proteomics Clin Appl. 2012; 6: 268-78. 408. van Balkom BW, Pisitkun T, Verhaar MC, Knepper MA. Exosomes and the kidney: prospects for diagnosis and therapy of renal diseases. Kidney Int. 2011; 80: 1138-45. 409. Miller RL, Sandoval PC, Pisitkun T, Knepper MA, Hoffert JD. Vasopressin inhibits apoptosis in renal collecting duct cells. Am J Physiol Renal Physiol. 2013; 304: F177-88. 410. Jensen TB, Pisitkun T, Hoffert JD, Jensen UB, Fenton RA, Praetorius HA, Knepper MA, Praetorius J. Assessment of the effect of 24-hour aldosterone administration on protein abundance in fluorescence-sorted mouse distal renal tubules by mass spectrometry. Nephron Physiol. 2012; 121: 9-15. 411. Saeed F, Pisitkun T, Knepper MA, Hoffert JD. An efficient algorithm for clustering of large-scale mass spectrometry data. IEEE Proceedings (IEEE Int Conf Bioinformatics Biomed). 2012; 4: 1-4. 412. Wilson JL, Miranda CA, Knepper MA. Vasopressin and the regulation of aquaporin-2. Clin Exp Nephrol. 2013; 17: 751-64. Review. 413. Yu P, Pisitkun T, Wang G, Wang R, Katagiri Y, Gucek M, Knepper MA, Geller HM. Global analysis of neuronal phosphoproteome regulation by chondroitin sulfate proteoglycans. PLoS One. 2013; 8: e59285. 414. Saeed F, Pisitkun T, Hoffert JD, Wang G, Gucek M, Knepper MA. An efficient dynamic programming algorithm for phosphorylation site assignment of large-scale mass spectrometry data. IEEE Proceedings (IEEE Int Conf Bioinformatics Biomed). 2012; 4: 618-625. 415. Knepper MA, Miranda CA. Urea channel inhibitors: a new functional class of aquaretics. Kidney Int. 2013; 83: 991-3. Comment. 416. Sandoval PC, Slentz DH, Pisitkun T, Saeed F, Hoffert JD, Knepper MA. Proteome-wide measurement of protein half-lives and translation rates in vasopressin-sensitive collecting duct cells. J Am Soc Nephrol. 2013; 24: 1793-805 417. Loo CS, Chen CW, Wang PJ, Chen PY, Lin SY, Khoo KH, Fenton RA, Knepper MA, Yu MJ. Quantitative apical membrane proteomics reveals vasopressin-induced actin dynamics in collecting duct cells. Proc Natl Acad Sci U S A. 2013; 10: 17119-24. 418. Claxton JS, Sandoval PC, Liu G, Chou CL, Hoffert JD, Knepper MA. Endogenous carbamylation of renal medullary proteins. PLoS One. 2013; 8: e82655.

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419. Saeed F, Pisitkun T, Hoffert JD, Rashidian S, Wang G, Gucek M, Knepper MA. PhosSA: Fast and accurate phosphorylation site assignment algorithm for mass spectrometry data. Proteome Sci. 2013; 11: S1-S14. 420. Hoffert JD, Pisitkun T, Saeed F, Wilson JL, Knepper MA. Global analysis of the effects of the V2 receptor antagonist satavaptan on protein phosphorylation in collecting duct. Am J Physiol Renal Physiol. 2014; 306: 410-21. 421. Saeed F, Hoffert JD, Knepper MA. CAMS-RS: Clustering algorithm for large-scale mass spectrometry data using restricted search space and intelligent random sampling. IEEE/ACM Trans Comput Biol Bioinform. 2014; 11: 128-41. 422. Miranda CA, Lee JW, Chou CL, Knepper MA. Tolvaptan as a tool in renal physiology. Am J Physiol Renal Physiol. 2014; 306: F359-66. 423. Knepper MA. Proteomic pearl diving versus systems biology in cell physiology. Am J Physiol Cell Physiol. 2014; 306: C634-5. 424. Bradford D, Raghuram V, Wilson JL, Chou CL, Hoffert JD, Knepper MA, Pisitkun T. Use of LC-MS/MS and Bayes' theorem to identify protein kinases that phosphorylate aquaporin-2 at Ser256. Am J Physiol Cell Physiol. 2014; 307: C123-39. 425. Trepiccione F, Pisitkun T, Hoffert JD, Poulsen SB, Capasso G, Nielsen S, Knepper MA, Fenton RA, Christensen BM. Early targets of lithium in rat kidney inner medullary collecting duct include p38 and ERK1/2. Kidney Int. 2014; 86: 757-67. 426. Knepper, MA, Raghuram V, Bradford D, Chou CL, Hoffert JD, Knepper MA, Pisitkun T. Systems biology versus reductionism in cell physiology. Am J Physiol Cell Physiol. 2014; 307: C308-9. 427. Saeed F, Hoffert JD, Pisitkun T, Knepper MA. Exploiting thread-level and instruction-level parallelism to cluster mass spectrometry data using multicore architectures. Netw Model Anal Health Inform Bioinform. 2014; 3:54 428. Grady CR, Knepper MA, Burg MB, Ferraris JD. Database of osmoregulated proteins in mammalian cells. Physiol Rep. 2014; 2: e12180. 429. Pisitkun T, Dummer P, Somparn P, Hirankarn N, Kopp JB, Knepper MA. Integrated design of antibodies for systems biology using Abdesigner. J Proteomics Bioinform. 2014; 7: 88-94. 430. Xie L, Subashi E, Qi Y, Knepper MA, Johnson GA. Four-dimensional MRI of renal function in the developing mouse. NMR Biomed. 2014; 7: 1094-102. 431. Sanghi A, Zaringhalam M, Corcoran CC, Saeed F, Hoffert JD, Sandoval P, Pisitkun T, Knepper MA. A knowledge base of vasopressin actions in the kidney. Am J Physiol Renal Physiol. 2014; 307: F747-55. 432. Yang CR, Tongyoo P, Emamian M, Sandoval PC, Raghuram V, Knepper MA. Deep

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proteomic profiling of vasopressin-sensitive collecting duct cells. I. Virtual western blots and molecular weight distributions. Am J Physiol Cell Physiol. 2015; 309: C785-98. 433. Yang CR, Raghuram V, Emamian M, Sandoval PC, Knepper MA. Deep proteomic profiling of vasopressin-sensitive collecting duct cells. II. Bioinformatic analysis of vasopressin signaling. Am J Physiol Cell Physiol. 2015; 309: C799-812. 434. Knepper MA, Kwon TH, Nielsen S. Molecular physiology of water balance. N Engl J Med. 2015; 372:1349-58. Review. 435. Knepper MA. Systems biology of diuretic resistance. J Clin Invest. 2015; 125:1793-5. Comment. 436. Lee JW, Chou CL, Knepper MA. Deep sequencing in microdissected renal tubules identifies nephron segment-specific transcriptomes. J Am Soc Nephrol. 2015; 26: 2669-77 437. Pickering CM, Grady CR, Medvar B, Emamian M, Sandoval PC, Zhao Y, Yang CR, Jung HJ, Chou CL, Knepper MA. Proteomic profiling of nuclear fractions from native renal inner medullary collecting duct cells. Physiol Genomics. 2015; physiolgenomics.00090.2015. PMID: 26508704. 438. Cheng L, Pisitkun T, Knepper MA, Hoffert JD. Peptide labeling using isobaric tagging reagents for quantitative phosphoproteomics. Methods Mol Biol. 2016;1355:53-70. Review. 439. Medvar B, Raghuram V, Pisitkun T, Sarkar A, Knepper MA. Comprehensive Database of Human E3 Ubiquitin Ligases: Application to Aquaporin-2 Regulation. Physiol Genomics. 2016 May 13, PMID: 27199454. 440. Huebner AR, Cheng L, Somparn P, Knepper MA, Fenton RA, Pisitkun T. Deubiquitylation of Protein Cargo Is Not an Essential Step in Exosome Formation. Mol Cell Proteomics. 2016; 15: 1556-71. PMID: 26884507. 441. Zhao Y, Yang CR, Raghuram V, Parulekar J, Knepper MA. BIG: a large-scale data integration tool for renal physiology. Am J Physiol Renal Physiol. 2016; 311: F787-F792. PMID: 27279488. 442. Sandoval PC, Claxton JS, Lee JW, Saeed F, Hoffert JD, Knepper MA. Systems-level analysis reveals selective regulation of Aqp2 gene expression by vasopressin. Sci Rep. 2016; 6:34863. PMID: 27725713. 443. LeMaire S, Raghuram V, Grady CR, Pickering CM, Chou CL, Umejiego EN, Knepper MA. Serine/Threonine Phosphatases and Aquaporin-2 Regulation in Renal Collecting Duct. Am J Physiol Renal Physiol. 2016: ajprenal.00455.2016 [Epub ahead of print] PMID: 27784696.