Aminoacylase 1 deficiency
Dec. 28, 2023
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Isonicotinic acid hydrazide is a hydrazine, as is the water-soluble toxin, monomethylhydrazine. Both isonicotinic acid hydrazide and monomethylhydrazine interfere with pyridoxine metabolism, which can result in GABA depletion and refractory seizures. Like monomethylhydrazine, isonicotinic acid hydrazide metabolites directly inactivate pyridoxine species, and isonicotinic acid hydrazide also inhibits pyridoxine phosphokinase. (Public domain.)