Q: About the finding of an abnormal glycogen


 

Question about Carbohydrate Metabolism (CM-12)

Glycogen structure

Glycogen structure

 

A liver biopsy is done on a child with hepatomegaly and mild fasting hypoglycemia. Hepatocytes show accumulation of glycogen granules with single glucose residues remaining at the branch points near the periphery of the granule. The most likely genetic defects is in the activity of:

 

a)     Alpha 1,6, glycosidase

 

b)     Alpha 1,4-alpha 1,4 glucan transferase

 

c)      Glucokinase

 

d)     Glucose 6 phosphatase

 

e)     Glucuronyl transferase

 

f)       Glycogen synthase

 

g)     Phosphoglucomutase

 

h)    UDP-glucose-Uridyl transferase

 

 

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