Lecture 27 - Glycogen Met. II Flashcards Preview

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Flashcards in Lecture 27 - Glycogen Met. II Deck (16):
1

End product of glycogenolysis

Glucose-1-P

2

Enzymes of glycogenolysis

1) Glycogenphosphorylase (GP)
2) Glycogen debranching enzyme (cleaves a-1,6)
3) Phosphoglucomutase (moves 3 sugars)

3

Branch point of glycogen is what kind of bond?

a-1,6

4

Lengths of glycogen bound by what type of bond?

a-1,4

5

Cleaves glycogen to form a G-1-P

Glycogenphosphorylase

6

Removes 3 glucose molecules near the a-1,6 branch point and places them on non-reducing end of another chain

transferase

7

Co-factor used in glycogenphosphatase

Vitamin B6

8

Cleaves the a-1,6 bond and removes the G-1-P by that bond

a-1,6 debranching enzyme (AKA "a-1,6 glucosidase")

9

Moves the glucose released by the a-1,6 debranching enzyme to another free end of glycogen.

transferase

10

Location of glucose-6-phosphatase

liver endoplasmic reticulum

11

Phosphoglucomutase

Converts G-1-P to G-6-P

12

Mutation to G-6-Pase in liver/kidney. Results in severe hypoglycemia, as glucose is not cleaved from glycogen.
A. GSD II Pompe
B. GSD V McArdle
C. GSD IV Hers
D. GSD I Von Gierke

D. GSD I Von Gierke

13

Mutation to muscle phosphorylase. Prevents the release of glucose from glycogen during heavy exercise, resulting in decreased muscle contraction.
A. GSD II Pompe
B. GSD V McArdle
C. GSD IV Hers
D. GSD I Von Gierke

B. GSD V McArdle

14

Mutation of liver phosphorylase. Causes severe hypoglycemia. Like Type I GSD, but mild.
A. GSD II Pompe
B. GSD V McArdle
C. GSD IV Hers
D. GSD I Von Gierke

C. GSD IV Hers

15

Mutation to alpha-1,4-glucosidase. Inability to cleave glycogen, causing severe hypoglycemia and increased glycogen stores. Usually fatal before 2 years of age.
A. GSD II Pompe
B. GSD V McArdle
C. GSD IV Hers
D. GSD I Von Gierke

A. GSD II Pompe

16

Deactivation of GS in the face of high glucose levels is accomplished via _____.

A. PFK
B. PKA
C. GSK
D. PP1

C. GSK (Addition of Pi to GS deactivates the enzyme, and stops glycogen synthesis)