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Cori\'s disease, a type llla glycogen storage disease, results in an enlarged li

ID: 92449 • Letter: C

Question

Cori's disease, a type llla glycogen storage disease, results in an enlarged liver in infants and muscle damage. These symptoms are a result of a mutation in an important enzyme in glycogen metabolism. When the liver cells are analyzed the glycogen present is only partially broken down and there are a large number of nonreducing ends remaining in the glycogen particle. What enzyme is defective in this disease? Glycogen synthase Phoshoglucomutase Debranching enzyme Branching enzyme Glucose 6-phosphatase Facilitated diffusion of a solute across a biological membrane is: driven by a difference of solute concentration. driven by the hydrolysis of ATP. endergonic. generally irreversible. not specific with respect to the substrate.

Explanation / Answer

Ans: deficiency of glycogen debraching enzyme results in cori disease. Symptoms include hypoglycemia and hypotonia.