Glycogen Metabolism Flashcards
What enzyme converts G6P to G1P?
Phosphoglucomutase
What enzyme activates glucose to form UDP-Glucose?
G1P UTP uridylyltransferase
What is glycogen branching enzyme?
A1;4,a1-6 Tranferase
Which end of the glycogen chain is attached to glycogenin?
Reducing end
What is the role of glycogen phosphorylase?
Phosphorylates and cleaves G1P from glycogen
What is debranching enzyme?
A1-4, a1-4 transferase
What enzyme removes the last glucose from a glycogen side chain?
Glucosidase
Describe Von Gierke Disease.
- Lack of G6Phospatase In ER Lumen
- Also could be protein which transports G6P from cytosol to ER
What enzyme is deficient in Pompe’s disease?
Lysosomal a-glucosidase
What enzyme is deficient in McArdles disease?
Skeletal muscle phosphorylase
What enzyme is deficient in Cory’s disease?
DeBranching enzyme
What enzyme is deficient in Andersons disease?
Defect in Branching enzyme
What enzyme is deficient in Hers disease?
Liver phosphorylase deficiency
What are the three key features which distinguish Diabetes vs Hyperlipidemia?
- Insulin/Glucagon levels
- Adipose tissue Lipase C not activated?
- Triglyceride accumulation in serum?