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Overview Function Pathway occurs in cytoplasm of all cells no ATP consumed or generated 2 phases oxidative produces NADPH glucose 6-phosphate (G6P) 6-phosphogluconate catalyzed by glucose 6-phosphate dehydrogenase (G6PDH) rate limiting step activated by NADP+, insulin inhibited by NADPH irreversible nonoxidative Clinical relevance glucose-6-phosphate dehydrogenase (G6PDH) deficiency pathophysiology NADPH production cells (specifically RBCs) lose protection against oxidizing agents cannot regenerate glutathione XR most common human enzyme deficiency prevalence among blacks malarial resistance by shortening the circulation life of RBCs Plasmodium does not have enough time for life span plasmodium does not have defense against free radicals in free radicals kills parasite presentation episodic hemolytic anemia intravascular hemolysis normocytic 2-3 days post precipitating stress foods fava beans common in Mediterranean foods presentation pallor, hemoglobinuria 24-48 post ingestion drugs sulfonamides, primaquine, antituberculosis drugs infection free radicals generated by the immune system Heinz bodies oxidized hemoglobin that precipitates within RBCs bite cells back pain test active hemolysis screen Heinz body prep Introduction The HMP shunt, also known as the pentose phosphate pathway, is a metabolic pathway that occurs in the cytoplasm of cells in most organisms