Neuromuscular

PATHWAYS: GLYCOGEN & GLUCOSE

Carbohydrate
Glucose to Pyruvate
  Animation
Glycogenolysis
Glycolysis
Glycogen disorders

Also see
  Lipid metabolism

From: R Brian Sommerville

Glycogenolysis Pathway

Glycogenolysis
  • Glycogen: Normal
    • Morphology: 20 nm particle
    • Composition
      • Glucose units
        • Chains: α-1,4-glucosidic linkage
        • Branches: α-1,6-glucosidic linkage
      • Protein core: Glycogenin
  • Polyglucosan
  • Glycogen Phosphorylase (PYGM)
    • Removes glucose residues from α-(1,4)-linkages within glycogen molecules
    • Product of reaction: Glucose-1-phosphate.
  • Phosphoglucomutase
    • Converts glucose-1-phosphate to glucose-6-phosphate.
    • Skeletal muscle: Glucose-6-phosphate is then metabolized by the glycolytic pathway
    • Diseases: CDG1t; GSD14
  • Phosphorylase b kinase
    • Reactivates phosphorylase by phosphorylating it
  • Debrancher enzyme
    • Removes glucose from branch points (α-(1,6)-linkages) within glycogen
  • Hexokinase

Glycolysis Pathway




Also see: MedicalBiochemistry
Progressive changes
Glycolysis of glucose to pyruvate.

A = Adenosine
P = Phosphate

From University of Virginia


Return to Neuromuscular Home Page
Return to Glycogen storage disorders

5/22/2020