Table 5.

Neurometabolic disorders with hyperammonemia and the involved enzymes

Neurometabolic disorder	Enzyme
Urea cycle defects	Carbamyl phosphate synthetase (CPS) deficiency, Ornithine transcarbamylase (OTC) deficiency, Argininosuccinate synthetase (AS) deficiency, Argininosuccinate lyase (AL) deficiency, Arginase deficiency?, Nacetyl glutamate synthetase deficiency
Organic acidemias	Propionic academia, Methylmalonic academia, Isovaleric academia, Beta-ketothiolase deficiency, Multiple carboxylase deficiency, Glutaric aciduria type II, 3-hydroxy -3-methylglutaric aciduria
Fatty acid oxidation disorders	Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD) Medium Chain Acyl CoA Dehydrogenase Deficiency (MCAD)