Von Geirke’s occurs due to deficiency of ?
**Core Concept**
Von Gierke's disease, also known as Glycogen Storage Disease Type I, is a congenital disorder caused by a deficiency of an enzyme involved in glycogen metabolism. This enzyme is crucial for the breakdown of glycogen to glucose in the liver.
**Why the Correct Answer is Right**
The correct answer is an enzyme called Glucose-6-phosphatase. This enzyme is necessary for the final step of glycogenolysis and gluconeogenesis, where glucose-6-phosphate is converted to glucose. Without this enzyme, glycogen accumulates in the liver cells, and the body is unable to maintain normal blood glucose levels. This leads to hypoglycemia, lactic acidosis, and other complications.
**Why Each Wrong Option is Incorrect**
* **Option A:** Glucose-6-phosphate dehydrogenase is an enzyme involved in the pentose phosphate pathway, not in glycogen metabolism.
* **Option B:** Phosphofructokinase is an enzyme involved in glycolysis, but its deficiency leads to a different disease called Glycogen Storage Disease Type VII.
* **Option C:** Glycogen synthase is an enzyme involved in glycogen synthesis, not in glycogen breakdown.
**Clinical Pearl / High-Yield Fact**
Von Gierke's disease is characterized by hypoglycemia, lactic acidosis, and hepatomegaly due to glycogen accumulation in the liver. It is an autosomal recessive disorder, and diagnosis is made by measuring enzyme activity in liver biopsy or blood samples.
**Correct Answer:** D. Glucose-6-phosphatase.