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What is The cause of hyperuricemia and gout in glucose-6- phosphatase deficiency is and explain in detail?
Purine overproduction and hyperuricemia in von Gierke disease (glucose-6-phosphatase deficiency) occurs secondary to enhanced generation of PRPP precursor, i.e. ribose-5-phosphate (a pentose).
In glucose-6-phosphatase deficiency, glucose-6-phosphate cannot be converted to glucose. Accumulated glucose-6-phosphate is then metabolized via HMP shunt, which in turn generates large amounts of ribose-5-phosphate, a precursor of PRPP. The increased synthesis of PRPP then enhances de novo synthesis of purine nucleotides.