Glycogen Metabolism Disorders

Question 1

Pneumonic for remembering glycogen disorder names?

Answer 1

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V = von Gierke's disease - Type 1
P = Pompe's disease - Type 2
C = Cori's disease - Type 3 and 3a
A = Andersen disease - Type 4
M = McArdle disease - Type 5
H = Hers' disease - Type 6

Question 2

What is Type 7 GSD called? What enzyme does it effect?

Answer 2

Tarui’s disease - m-PFK-1

Question 3

What enzyme does type 9 GSD affect?

Answer 3

Phosphorylase kinase (PHOK) of liver / muscle, enzyme responsible for activating glycogen phosphorylase (in glycogenolysis)

Question 4

What enzyme affects type 1 GSD and how do you remember?

Answer 4

Glucose-6-phosphatase - liver enzyme only. von Gierke’s and the enzyme both have a G and no Y!

Question 5

What enzyme affects type 2 GSD and how do you remember?

Answer 5

acid alpha-glucosidase, in muscle lysosomes only. Remember this because Pompe’s disease sounds like Pompeii, and acid / fiery death = acid alpha-glucosidase

Question 6

What pneumonic tells you the enzymes for Type 3-4 GSD?

Answer 6

ABCD - affect liver

Anderson - Branching enzyme

Cori’s - Debranching enzyme

3a is both muscle and liver.

Question 7

How you remember the type 5 enzyme defect?

Answer 7

McArdle disease = M

M means Muscle phosphorylase (glycogen phosphorylase in muscle)

Question 8

How do you remember the type 6 enzyme defect?

Answer 8

Hers’ disease

LivHER - Liver phosphorylase (glycogen phosphorylase in liver)