seizure is a rare presentation for acute hemolysis due to g6pd deficiency. we report a previously healthy boy who presented initially with seizure and cyanosis and subsequently acute hemolysis, due to glucose-6-phosphate dehydrogenase deficiency (g6pd) and probably secondary methemoglobinemia, following the ingestion of fava beans.

Low concentrations of palmitoyl coenzyme A inhibit yeast glucose-6-P dehydrogenase (TAKETA, K., AND POGELL, B. M. (1966) J. Biol. Chem. 241, 720). This inhibition is prevented or reversed by bovine serum albumin, mycobacterial polysaccharides, or alkylated cyclodextrins. Sephadex chromatography and sucrose density gradient centrifugation show that palmitoyl-CoA inhibits by dissociating the tetr...

Homogeneous biosynthetic sn-glycerol-3-phosphate dehydrogenase (EC 1.1.1.8) of Escherichia coli was potently inhibited by palmitoyl-CoA and other long chain acyl-CoA thioesters. The concentration dependence of this inhibition was not cooperative. Enzyme activity was inhibited 50% at 1 microM palmitoyl-CoA; thus, this inhibition occurred at concentrations below the critical micellar concentratio...