Q: About GSD and Glycogen structure (C-09)


 

Andersen’s Disease is a rare disease caused by the deficit of the Branching enzyme, responsible for the formation of branches in glycogen structure. This results in an abnormal glycogen with few branching points and long peripheral chains. Clinically, hepatoesplenomegaly, cirrhosis of the liver and hepatic failure are major concerns. As the problem is located in the branching points, it is obvious that there is a defect in the formation of:

 

a)     Alpha 1,4 -O-glycosidic linkages

 

b)     Alpha 1,6 -O-glycosidic linkages

 

c)      Alpha 1 ,Beta 2 -O-glycosidic linkages

 

d)     Beta1,3 -O-glycosidic linkages

 

e)     Beta1,4 -O-glycosidic linkages

 

f)       Beta1,6 -O-glycosidic linkages

 

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