ENZYMESOF LIPID MET ABOLISM

ADVANCES IN EXPERIMENTAL MEDICINE AND BIOLOGY Editorial Board:

Nathan Back State University 0/ New Y ork at Buf/alo

N. R. Di Luzio Tulane University School 0/ Medicine

Bernard Halpern College de France and Institute o/Immuno·Biology

Ephraim Katehalski The Weizmann Institute 0/ Science

David Kritchevsky Wistar If'stitute

Abel Lajtha New York State Research Institute tor Neurochemistry and Drug Addiction

Rodolfo Paoletti University 0/ Milan

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Edited by Shimon Gatt, Louis Freysz, and Paul Mandel

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Edited by H. James Day, Basil A. Molony, Edward E. Nishizawa, and Ronald H. Rynbrandt

ENZYMES OF LIPID METABOLISM

Edited hy

Shimon Gatt Hebrew University - Hadassah Medical School Jerusalern, Israel

Louis Freysz C.N.R.S. Center for Neurochernistry Louis Pasteur University Strasbourg, France

and

Paul Mandel C.N.R.S. Center for Neurochernistry Louis Pasteur University Strasbourg, France

PLENUM PRESS • NEW YORK AND LONDON

Library of Congress Cataloging in Publication Data

Main entry under title:

Enzymes of lipid metabolism.

(Advances in experimental medicine and biology; v. 101) Proceedings. Includes index. 1. Lipid metabolism-Congresses. 2. Enzymes-Congresses. I. Gatt, Shimon. 11. Freysz,

Louis. III. Mandel, Paul. IV. France. Centre national de la recherche scientifique. [DNLM: 1. Lipids-Metabolism-Congresses. 2. Enzymes-Metabolism-Congresses. QU85 E611977] QP751.E59 599'.01'9247 78-886 ISBN 978-1-4615-9073-6 ISBN 978-1-4615-9071-2 (eBook) DOI 10.1007/978-1-4615-9071-2

Proceedings of a Colloquium sponsored by C.N.R.S. held in Strasbourg, France, April 18-22, 1977

Organizers

Shimon Gatt and Paul Mandel

General Secretary

L. Freysz

Organizing Committee

H. Dreyfus A. Farooqui N. Neskovic G. Rebel J. Robert L. Sarlieve G. Vincendon

Scientifie Advisory Committee

P. Desnuelle J. Polonovski K. Suzuki L. Svennerholm G. Tettamanti

© 1978 Plenum Press, New Y ork Softcover reprint of the hardcover 1st edition 1978 A Division of Plenum Publishing Corporation 227 West 17th Street, New York, N.Y.l00n

All rights reserved

No part of this book may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, microfilming, recording or otherwise, without written permission from the Publisher

Preface

This book presents the proceedings of the meeting on "Enzymes of Lipid Metabolism" which took place April 18-22, 1977, at the Mont Ste. Odile convent in Alsace, France. The meeting was convened and co-ehaired by P. Mandel and S. Gatt. L. Freysz was general seeretary. They were assisted by an international advisory commit­tee and a loeal organizing committee.

The purpose of the meeting was to bring together scientists involved in research on a wide variety of aspeets of lipid metabo­lism and who have a direct interest in the problematies on inter­acting enzymes with lipid substrates. The aim was to emphasize the latter aspect rather than common enzymologieal aspects such as extraetion, purification, and protein strueture. The rpeoeedings included several symposia and four "round-table" discussions. There was also a presentation of posters, whieh were exhibited during the entire duration of the meeting. All partieipants who presented leetures or posters were requested to summarize their findings, whieh were ineluded in the book. Regretfully, the very fruitful discus­sions of the "round-tables" were not included, nor were those held in the informal atmosphere of the court yards of the eonvent, the hiking paths in the Vosges mountains, and the bar. For the purpose of coherence, the editors decided not to adhere to the original order of the lectures. The papers in the book are grouped ae­eording to the classes of enzymes and the types of reaction which they catalyze.

The very generous assistance of the CNRS, who sponsored the colloquium, is gratefully acknowledged. Without the generous grant of this ageney, the meeting would not have materialized.

We wish to thank members of the scientific advisory committee and loeal organizing committee, as well as Mrs. H. Urban artd M. Sarlieve for excellent secretarial assistance.

v

S. Gatt L. Freysz P. Mandel

Contents

Enzymes Metabolizing Phospholipids: From Infancy to Middle Age (Plenary Lecture) •••••

R.M.C. Dawson

ENZYMES OF THE METABOLISM OF FATTY ACIDS AND NEUTRAL GLYCERIDES

Fatty Acid Biosynthesis during Brain Development J.M. Bourre, S. Pollet, M. Paturneau-Jouas, and N. Baumann

The Role of Soluble Acyl-Thioester Hydrolase in Fatty Acid Chain-Length Termination in Rabbit Mammary Gland and Liver • • • • • • • • • • • • •

J. Knudsen, L. Chivers, and R. Dils

Stereochemical Studies of Hydrogen Incorporation from Nucleotides with Fatty Acid Synthetase from Brevibacterium ammoniagenes • • • • • . • • • •

Y. Seyama, T. Kasama, T. Yamakawa, A. Kawaguchi, and S. Okuda

Cholesterol Oxidase as a Probe for Studying Membrane

1

17

27

37

Composition and Organization • • • • 45 Y. Barenholz, E.J. Patzer, N.F. Moore, and R.R. Wagner

Adsorption and Activation of Pancreatic Lipase at Interfaces • • • • • • • • . • • • •

C. Chapus, M. Semeriva, M. Charles, and P. Desnuelle

Mode of Action of Pancreatic Colipase • • . • B. Borgström

Studies of Lipase and Phospholipase A2 Acting on Lipid Monolayers . • • • • • • • • • • • .

R. Verger, J. Rietsch, F. Pattus, F. Ferrato, G. Pieroni, G.H. de Haas, and P. Desnuelle

vii

57

69

79

viii CONTENTS

Inhibition of Lipase Adsorption at Interfaces Role of Bile Salts MicelIes and Colipase • • • • •• 95

D. Lairon, G. Nalbone, H. Lafont, J. Leonardi, N. Domingo, J.C. Hauton, and R. Verger

Lipases of Rat Brain Microsomes M.C. Cabot and S. Gatt

Identification and Some Characteristics of the Enzyme Protein of the Hormone-Sensitive Lipase from Rat Adipose Tissue

P. Belfrage, B. Jergil, P. Strälfors, and H. Tornqvist

Affinity Chromatography on Heparin-Sepharose of Rat Adipose Tissue Triglyceride Lipase from Cytosol • • • • • • •

A. Vanhove, M. Breton, and J. Polonovski

ENZYMES OF THE METABOLISM OF GLYCEROPHOSPHOLIPIDS

A) Phospholipases

Relationship between Structure and Activity of

101

113

127

Pancreatic Phospholipase A2 •••• • • • •• 137 A.J. Slotboom, M.C.E. van Dam-Mieras, E.H.J.M. Jansen, F. Pattus, H.M. Verheij, and G.H. de Haas

Kinetic Anomalies Associated with Phospholipase A2 Hydrolysis of Micellar Substrates • • • • •• •• 153

T.T. Allgyer and M.A. Wells

Mechanism of Phospholipase A2 Action Towards Mixed MicelIes of Detergent and Phospholipids

E.A. Dennis 165

Chemical Modification of Crotalus atrox Phospholipase A2 by Means of a Photolabile Phosphatidylethano-lamine Analog • • • • • • • • • • • • • • • • • • 177

K.-S. Huang and J.H. Law

Phospholipases A of Krebs 11 Ascites Cells: Release and Subcellular Localization

J. Lloveras, M. Record, G. Ribbes, and L. Douste-Blazy

Specificity, 185

CONTENTS

Phospholipases of Plasmic Membranes of Adipose Tissue. Possible Intermediaries for Insulin Action • • • • • • • •

G. Bereziat, C. Wolf, O. Colard, and J. Polonovski

Calcium-Activated Hydrolysis of Triphosphoinositide in Synaptosomes ••••••••••••••

J.N. Hawthorne and H.D. Griffin

The Activity of Phospholipase D on Aggregates of Phosphatidylcholine, Dodecylsulfate and Ca2+

M. Heller, P. Greenzaid, and D. Lichtenberg

The Preparation of Phospholipids by Phospholipase D S. Kovatchev and H. Eibl

The Effects of Amphiphilic Compounds on Phosphatidate Metabolism • • • • • • • • • • • • • • .

D.N. Brindley, M. Bowley, R.G. Sturton, P.H. Pritchard, J. Cooling, and S.L. Burditt

B) Biosynthesis

Choline and Ethanolamine Kinase Activity in the Cytoplasm of Nerve Endings from Rat Forebrain

S. Spanner and G.B. AnseIl

The Release of Free Ethanolamine in Rat Brain Homogenates Incubated in Krebs Ringer •

S. Spanner and G.B. AnseIl

Ethanolamine and Choline Phosphotransferases of Chicken Brain • • • • • • • • • • • •

L. Freysz, L.A. Horrocks, and P. Mandel

Studies of Rat Brain Choline Ethanolamine Phosphotransferases Using Labeled Alkylacylglycerol as Substrate with Evidence for Reversibility of

191

201

213

221

227

237

247

253

the Reactions • • • •• • • • • • • • • • • • •• 269 G. Goracci, L.A. Horrocks, and G. Porcellati

The Role of Phosphatidylglycerol as a Donor of Phosphatidyl and of ~-Glycerol-l-Phosphate Groups in Biosynthetic Reactions • • • • •

R.A. Pieringer, J.M. Shaw, and M.-C.W. Ganfield 279

ix

x

Membrane-Bound Base-Exchange Reactions in Anima1 Tissues . • . • • • • • • • . • . • • • • •

G. Porce11ati, A. Gaiti, H. Woe1k, G.E. De Medio, M. Brunetti, E. Francescange1i, and G. Trovare11i

The "Base-Exchange" Reaction: The Serine Enzyme T. Taki, T. Miura, and J.N. Kanfer

A Study on the Possib1e Occurrence of Base-Exchange Reactions in Vivo • • • • • • • • • • • • • • ---P. Or1ando, G. Arienti, P. Saracino, L. Corazzi,

P. Massari, and G. Porce11ati

The Biosynthesis and Hormonal Regulation of Surfactant Formation • • • • • •

J.M. Johnston, J.C. Porter, and P.C. MacDona1d

Properties of a Lysophospho1ipase-Transacy1ase from Rat Lung: lts Possib1e Invo1vement in the Synthesis of Lung Surfactant •• • • •

H. van den Bosch and G. W. Brum1ey

A Study on the Turnover of Rat Brain Phosphatidic Acid through the G1ycero1-Phosphate Pathway

L. Binag1ia, R. Roberti, and G. Porce11ati

C) Ether Lipids

Studies on the Biosynthesis of the O-A1ky1 Bond in G1ycero1 Ether Lipids

A.K. Hajra, C.L. Jones, and P.A. Davis

Asymmetrie Loca1ization of A1kyldihydroxyacetone-P Synthase and Acyldihydroxyacetone-P Acyltransferase in Microsoma1 Vesicles

C.O. Rock and F. Snyder

Studies on Soluble Proteins Stimu1ating Plama10gen Biosynthesis • • • • •

F. Paltauf

CONTENTS

287

301

319

327

341

353

369

379

387

Pathway for Hydrolysis of Plasmalogens L.A. Horrocks and S.C. Fu

in Brain . . . . . . . 397

Turnover of Mo1ecular Species of Diacyl and Alkyl Ether Phospho1ipids in Ehrlich Ascites Tumor Ce11s • • • • • • • • • • • • • • • • • 407

K. Waku and Y. Nakazawa

CONTENTS

ENZYMES OF THE METABOL1SM OF SPH1NGOL1P1DS

A) Neuraminidases

Neuraminidase Gang1iosides Interactions • G. Tettamanti, B. Cestaro, B. Venerando, and A. Preti

Action of Rat Brain Sia1idase on Synaptic Membrane Components in situ • • • •

A. Rosenberg

Interaction of Human Brain Neuraminidase with Tritium-Labe11ed Gang1iosides ••••

R.W. Veh and R. Schauer

Studies on Bovine Brain Membrane-Bound

417

439

447

Neuraminidase (Sia1idase) • • • • • • • • • • • • • •• 463 K. Sandhoff, P. Pa11mann, H. Wiegandt, and W. Zieg1er

Intraneuronal Distribution of Cytoso1ub1e Neuraminidase in Pig Brain • • • • • •

B. Venerando, A. Preti, A. Lombardo, B. Cestaro, V. Zambotti, and G. Tettamanti

B) Sphingomye1inases

Effect of Detergents on the Uti1ization of Sphingomye1in by Brain Sphingomye1inases

S. Gatt, T. Dinur, S. Yedgar, and Z. Leibovitz-Ben Gershon

Sphingomye1inase of Human Tissues S. Yamaguchi and K. Suzuki

C) G1yco1ipid Hydro1ases

Purification and Partial Characterization of Human Liver a-Ga1actosidase: 1s a-Ga1actosidase B an a-N-Acety1ga1actosaminidase? . • •

K.J. Dean, S.S.J. Sung, and C.C. Swee1ey

The 1dentity of a-Ga1actosidase B from Liver A.W. Schram, M.N. Hamers, and J.M. Tager

475

487

501

515

525

xii

Effect of Detergents on Ceramide-3 Hydrolysis by a-Ga1actosidase A • • • • • • • • • •

A.W. Schram, M.W. Ho, S. Cordus, M. Samson, M.N. Hamers, J. Brown, D. Robinson, and J.M. Tager

Specificity of Some Exo- and Endo-ß-Ga1actosidases toward G1ycosphingo1ipids • •

Y.-T. Li, C.C. Wan,J.-L. Chien, and S.-C. Li

Studies of Activity and Stabi1ity of Immobi1ized ß-N-Acety1hexosaminidase • • • •

K.-K. Yeung, A.J. Owen, and J.A. Dain

Affinity Methods for the Purification of Ary1su1phatase A • • • • •

G. Dubois and N. Baumann

Existence, Localization and Some Properties of the Activators of Sphingo1ipid Hydro1ases • • • •

H. Jatzkewitz

Studies on the Function of the Activator of Su1phatase A • • • • • • • • • •

G. Fischer and H. Jatzkewitz

G1ucosidase and G1ucocerebrosidase Activity in Subce11u1ar Fractions of Rat Spleen •

Z. Leibovitz-Ben Gershon, J. Rosentha1, and A. Russe11

Inhibitors of ß-G1ucosidases of Anima1 Tissues N.S. Radin

Isolation of Lysosomes from Bovine Brain Tissue. A New Zonal Centrifugation Technique • • • •

B. Overdijk, G.J.M. Hooghwinke1, and J.J.W. Lisman

D) Biosynthesis

UDP-Ga1actose:Ceramide Ga1actosy1transferase. Properties and Effect of Detergents and Phospho1ipids on the Partia11y Purified of Rat Brain •• • • • • • •

N.M. Neskovic, P. Mandel, and S. Gatt

Kinetic

Enzyme

Isolation of an Endogenous Inhibitor of Ceramide G1ycosy1transferases from Rat Brain • • • •

E. Costantino-Ceccarini, A. Cestelli, and K. Suzuki

CONTENTS

531

537

545

555

561

573

583

589

601

613

631

CONTENTS

Ce11u1ar Loca1ization of Na+ ~-Dependent ATPase and of the Enzymes of Su1pho1ipid Metabo1ism in Rabbit Kidney • • • • • • • • • • • • •

J.J. He1wig, J. Pieringer, L.L. Sar1ieve, A.A. Farooqui, G. Rebe1, P. Mandel, and R.A. Pieringer

Contro1 of Gang1ioside Biosynthesis by a Rate Limiting UMP-ase and Product Gang1ioside Inhibition • . • • • • • • • • • • •

J.A. Dain and W.R. Hitchener

Studies on Retina1 Gang1ioside Metabo1ism H. Dreyfus, S. Harth, A. Preti, P.F. Urban, and P. Mandel

Characteristics of the Brain Tissue Disia1ogang1ioside G2a

D. Kostic

Formation of G1ycosy1ated "Proteo1ipid-Like" Materials during Exogenous Protein Ga1actosy1ation

xiii

641

649

655

667

by Human Body Fluids • • • • • • • • • • • • • 679 J.A. Dain and E. Raghupathy

ENZYMATIC ASPECTS OF THE LIPID STORAGE DISEASES

Diagnoses of the Sphingo1ipidoses with Labe11ed Natural Substrates •••••••••••

L. Svennerho1m

Assay of ß-G1ucosidase and Sphingomye1inase for Identification of Patients and Carriers of Gaucher's and Niemann Pick Diseases ••••

D.A. Wenger

Hair Root Analysis in Heterozygotes for Fabry's Disease ••••••••••••••

A. Ejiofor, D. Robinson, D. Wise, M.N. Hamers, and J.M. Tager

Substrate Specificity of Hexosaminidase A Iso1ated from the Liver of a Patient with a Rare Form (AB Variant) of Infantile GM2 Gang1iosidosis and Contro1 Tissues • • • • • • • • • • •

K. Sandhoff, E. Conze1mann, and H. Nehrkorn

Biochemica1 Studies in Sphingomye1in Storage Disorders M.T. Vanier

689

707

719

727

731

xiv

Faetors that Influenee the Uptake and Turnover of Glueoeerebrosidase and a-Galaetosidase in Mammalian Liver • • • • • • • • • • • • • •

P.G. Pentehev, J.W. Kusiak, J.A. Barranger, F.S. Furbish, S.I. Rapoport, J.M. Massey, and R.O. Brady

Enzyme Therapy XI: Immunologie Considerations for Replaeement Therapy with Unentrapped, Erythroeyte-

CONTENTS

745

and Liposome-Entrapped Enzymes •••••• • • • • 753 R.J. Desniek, M.B. Fiddler, S.D. Douglas, and L.D.S. Hudson

List of Partieipants 765

Contributor Index • 773

Subjeet Index 777