Glucose-6-phosphatase Deficiency and Glycogen Storage Diseases

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Flashcards about Glucose-6-phosphatase Deficiency and Glycogen Storage Diseases

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1
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Which enzyme deficiency causes Von Gierke's disease (Glycogen storage disease type Ia)?

Glucose-6-phosphatase

2
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What is the role of Glucose-6-phosphatase?

It plays a critical role in gluconeogenesis and glycogenolysis by catalyzing the conversion of glucose-6-phosphate to glucose.

3
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What are the principal metabolic effects of glucose-6-phosphatase deficiency?

Hypoglycemia, lactic acidosis, hypertriglyceridemia, and hyperuricemia.

4
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Which metabolic mechanism is affected by glucose-6-phosphatase deficiency?

Glycogenolysis

5
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In which Glycogen storage disease is Debranching enzyme deficient?

Cori Disease

6
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In which Glycogen storage disease is Branching enzyme deficient?

Andersen Disease

7
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In which Glycogen storage disease is Myophosphorylase deficient?

McArdle Disease