Biochemistry · Carbohydrate Metabolism (Glycolysis, Gluconeogenesis, Glycogen, HMP Shunt)

Phosphoglucose isomerase (PGI) deficiency presents with hemolytic anemia without hypoglycemia. The hemolysis occurs because RBCs depend exclusively on which pathway for ATP, and PGI deficiency traps glucose-6-phosphate upstream?

  • A HMP shunt producing NADPH
  • B Glucuronic acid pathway
  • C Embden-Meyerhof glycolysis producing ATP
  • D Galactose oxidation pathway
Correct answer: C. Embden-Meyerhof glycolysis producing ATP

Explanation

Mature RBCs lack mitochondria and depend entirely on the Embden-Meyerhof (anaerobic) glycolytic pathway for ATP production. PGI converts G6P to fructose-6-phosphate; its deficiency blocks glycolytic flux, depleting ATP in RBCs causing membrane failure and hemolysis. There is no hypoglycemia because the liver, which has the complete metabolic toolkit, compensates readily.

Reference: Harper's Illustrated Biochemistry, 32nd ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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