In VonGierke’s disease, the levels of ketone bodies are increased due to all EXCEPT:
Correct Answer: OAA is required for gluconeogenesis
Description: Type I Glycogen storage disease is known as Von Gierke's disease. Enzyme deficient is Glucose-6-Phosphatase. This enzyme is common for two pathways - Glycogenolysis & Gluconeogenesis. Therefore in type I disease, both glycogenolysis & gluconeogenesis cannot occur leading to very severe hypoglycemia & ketosis occurs because of severe hypoglycemia. Ketone body synthesis in starvation vs Von Gierke's disease: In starvation in starvation - Gluconeogenesis is| - removal of OAA for gluconeogenesis - TCA cycle hampered - acetyl-CoA builds up - ketone body synthesis| So ketosis occur due to lack of OAA. In von Gierke's disease Gluconeogenesis and glycogenolysis not occurring due to lack of common enzyme - Hypoglycemia - ketone body synthesis| So ketosis occurs due to hypoglycemia and not due to lack of OAA. hence the reason for increased ketone body synthesis in Von Gierke's disease is not the "use of OAA for Gluconeogenesis". Hence Option D is a wrong statement and is the Answer.
Category:
Biochemistry
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