Target intelligence / Profile preview

Glycogen Debranching Enzyme (None)

Target
None
Molecular classification
Enzyme, Glycoside Hydrolase, Transferase, Glucosidase
01

Overview

Glycogen debranching enzyme (GDE) is a crucial enzyme in glycogen metabolism, responsible for the breakdown of branched glycogen molecules to release glucose. It is a multifunctional, bifunctional enzyme with two distinct catalytic activities: transferase and glucosidase. GDE works alongside glycogen phosphorylase to mobilize glucose from glycogen stores by transferring three glucose residues from the branch to another chain and cleaving the remaining α-1,6 linkage to release free glucose. Deficiency or mutations in AGL cause glycogen storage disease type III (GSDIII), characterized by hepatomegaly, hypoglycemia, and myopathy.

Other names
GDEAmylo-α-1,6-GlucosidaseOligo-1,4-α-GlucanotransferaseAGL
02

Mechanism of action

Inhibition of glycogen debranching enzyme activity (GDE) to modulate glucose release from glycogen stores. Currently, there are no known drugs that target GDE. The mechanism would be inhibition to slow down glycogen breakdown in certain diseases.

03

Biological functions

GlycogenolysisGlucose metabolismCarbohydrate metabolism
04

Disease associations

Glycogen Storage Disease Type III (GSDIII)Cori DiseaseForbes Disease
05

Safety considerations

HypoglycemiaHepatomegalyMyopathy
06

Biomarkers

Elevated liver enzymes (ALT, AST)HypoglycemiaHepatomegalyMuscle weaknessGlycogen accumulation in liver and muscle tissues

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