glycogen synthesis
DESCRIPTION
Berge Minassian, M.D.TRANSCRIPT
![Page 1: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/1.jpg)
![Page 2: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/2.jpg)
![Page 3: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/3.jpg)
![Page 4: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/4.jpg)
![Page 5: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/5.jpg)
![Page 6: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/6.jpg)
Glycogen synthase
Glycogen branching enzyme
Glycogen Synthesis
![Page 7: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/7.jpg)
![Page 8: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/8.jpg)
GS ~ BE
![Page 9: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/9.jpg)
![Page 10: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/10.jpg)
↑GS Or ↓BE
![Page 11: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/11.jpg)
↑GS Or ↓BE
![Page 12: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/12.jpg)
![Page 13: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/13.jpg)
Adult Polyglucosan Body Disease ↓BE
Lafora Disease ↑GS
![Page 14: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/14.jpg)
GS ~ BE
![Page 15: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/15.jpg)
GS ~ BE
![Page 16: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/16.jpg)
GS
PP1
PTG
GS
![Page 17: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/17.jpg)
PTG -/-Laforin -/-
X
PTG -/- Laforin -/-
![Page 18: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/18.jpg)
0
0.05
0.1
0.15
0.2
0.25
0.3
1
LKODKO
![Page 19: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/19.jpg)
![Page 20: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/20.jpg)
![Page 21: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/21.jpg)
![Page 22: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/22.jpg)
![Page 23: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/23.jpg)
![Page 24: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/24.jpg)
![Page 25: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/25.jpg)
![Page 26: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/26.jpg)
GS ~ BE
![Page 27: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/27.jpg)
![Page 28: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/28.jpg)
PTG -/-Malin -/-
X
PTG -/- Malin -/-
![Page 29: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/29.jpg)
![Page 30: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/30.jpg)
Adult Polyglucosan Body DiseaseLafora Disease
![Page 31: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/31.jpg)
Glycogen synthase
![Page 32: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/32.jpg)
Glycogen synthase
![Page 33: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/33.jpg)
Adult Polyglucosan Body Disease
![Page 34: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/34.jpg)
GS ~ BE
![Page 35: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/35.jpg)
PTG -/-GBE1 -/-
X
PTG -/- GBE1 -/-
![Page 36: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/36.jpg)
Glycogen synthaseSmall molecule
siRNA
![Page 37: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/37.jpg)
![Page 38: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/38.jpg)
![Page 39: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/39.jpg)
![Page 40: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/40.jpg)
![Page 41: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/41.jpg)
Amylase +/+GBE1 -/-
X
Amylase +/+ GBE1 -/-
![Page 42: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/42.jpg)
![Page 43: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/43.jpg)
![Page 44: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/44.jpg)
![Page 45: Glycogen Synthesis](https://reader038.vdocuments.mx/reader038/viewer/2022102722/5562f6d5d8b42a62598b472c/html5/thumbnails/45.jpg)
SummaryExperiment I: GBE-/- x
PTG-/-
Will prove whether downregulating GS STOPS and PREVENTS APBD.
Will focus subsequent work on finding a small molecule that interferes with and slows GS activity.
Polyglucosan accumulation is probably a NET accumulation, i.e. the difference between their production and elimination. Potentially, stopping production could result in natural gradual elimination, and cure.
Experiment II: GBE-/- x Amylase +/+
Will prove whether introducing amylase into neurons clears polyglucosans and cures APBD.
Will focus subsequent work on finding a way to introduce amylase into the brain, ONLY ONCE. Single introduction should clear polyglucosans accumulated for 50 years and thus allow freedom from disease for the next 50 years.
Experiment III: Introducing amylase (or other) across the blood brain barrier
This experiment would be the way to actually apply the previous experiment, and introduce amylase into the brain and neurons and eliminate polyglucosan bodies.