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A patient was seen by a gastrointestinal specialist and diagnosed with a glycogen storage disease. A...

A patient was seen by a gastrointestinal specialist and diagnosed with a glycogen storage disease. A liver biopsy showed that the patient was producing very little glycogen, the molecules were relatively small, and they only had a(1,4) glycosidic linkages. Blood and liver glucose concentrations were within normal ranges. What enzyme is likely affected in this disease?

a. glycogen synthase

b. glycogen phosphorylate

c. branching enzyme

d. debranching enzyme

e. hexokinase

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Answer #1

The answer is the 'debranching enzyme'.

Explanation: People with glycogen storage disease don’t have enough of an enzyme called the debranching enzyme, which helps in breaking down the glycogen. The glycogen can’t fully break down. It collects in the liver and in muscle tissues. The symptoms include a swollen belly, delayed growth, and weak muscles.

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