Glycogen storage disorders

Glycogen storage disease which presents as lysosomal disease​ ​ (a)  

• Pompe's disease
• von Gierke's disease
• Core's disease
• Andersons disease

Glycogen storge disease with predominant muscle involvement

A young female is found to have hepatomegaly, hypoglycemia, elevated levels of acetone. Biochemical testing reveals deficiency of liver phosphorylase .

• Cori's disease

• Pompe's disease

• Tauris disease

• Hers disease

A 6 month old baby brought to the emergency department with features of cardiac failure. On examination, she found to have developmental delay and short stature . The most probable diagnosis _​ (a)  

• Pompe's disease
• McArdles disease
• Andeson's disease

A child brought to the hospital found to have mild hypoglycemia, hepatomegaly and accumulation of highly branched limit dextrins. He is likely to be suffering from

• von Gierke's disease

• Cori's disease

• McArldes disease

• Anderson's disease

A patient presents with von Gierke's disease and ketosis was detected on investigation. All of the following would be associated findings EXCEPT

• Low plasma glucose level

• Hepatospenomegaly

• Impaired gluconeogenesis

• Low fat mobilization

Glycogen phosphorylase regulated by aa EXCEPT

• cAMP

• Calmodulin

• Glycogenenin

• Protein kinase A

The cofactor for glycogen phosphorylase is

• B1

• B2

• B6

• B9

Glycogen phosphorylase is an example for

• Oxidoreductase

• Transferase

• Hydrolase

• Lyase