Glycogen Synthesis
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Transcript of Glycogen Synthesis
Glycogen synthase
Glycogen branching enzyme
Glycogen Synthesis
GS ~ BE
↑GS Or ↓BE
↑GS Or ↓BE
Adult Polyglucosan Body Disease ↓BE
Lafora Disease ↑GS
GS ~ BE
GS ~ BE
GS
PP1
PTG
GS
PTG -/-Laforin -/-
X
PTG -/- Laforin -/-
0
0.05
0.1
0.15
0.2
0.25
0.3
1
LKODKO
GS ~ BE
PTG -/-Malin -/-
X
PTG -/- Malin -/-
Adult Polyglucosan Body DiseaseLafora Disease
Glycogen synthase
Glycogen synthase
Adult Polyglucosan Body Disease
GS ~ BE
PTG -/-GBE1 -/-
X
PTG -/- GBE1 -/-
Glycogen synthaseSmall molecule
siRNA
Amylase +/+GBE1 -/-
X
Amylase +/+ GBE1 -/-
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.