Glycogen Metabolism

Question 1

What enzyme converts G6P to G1P?

Answer 1

Phosphoglucomutase

Question 2

What enzyme activates glucose to form UDP-Glucose?

Answer 2

G1P UTP uridylyltransferase

Question 3

What is glycogen branching enzyme?

Answer 3

A1;4,a1-6 Tranferase

Question 4

Which end of the glycogen chain is attached to glycogenin?

Answer 4

Reducing end

Question 5

What is the role of glycogen phosphorylase?

Answer 5

Phosphorylates and cleaves G1P from glycogen

Question 6

What is debranching enzyme?

Answer 6

A1-4, a1-4 transferase

Question 7

What enzyme removes the last glucose from a glycogen side chain?

Answer 7

Glucosidase

Question 8

Describe Von Gierke Disease.

Answer 8

• Lack of G6Phospatase In ER Lumen

- Also could be protein which transports G6P from cytosol to ER

Question 9

What enzyme is deficient in Pompe’s disease?

Answer 9

Lysosomal a-glucosidase

Question 10

What enzyme is deficient in McArdles disease?

Answer 10

Skeletal muscle phosphorylase

Question 11

What enzyme is deficient in Cory’s disease?

Answer 11

DeBranching enzyme

Question 12

What enzyme is deficient in Andersons disease?

Answer 12

Defect in Branching enzyme

Question 13

What enzyme is deficient in Hers disease?

Answer 13

Liver phosphorylase deficiency

Question 14

What are the three key features which distinguish Diabetes vs Hyperlipidemia?

Answer 14

• Insulin/Glucagon levels
• Adipose tissue Lipase C not activated?
• Triglyceride accumulation in serum?