Nudix hydrolase 8 (NUDT8)

Nudix hydrolase 8 (NUDT8) is a mitochondrial enzyme belonging to the nudix hydrolase family that functions as an acyl-CoA diphosphatase, catalyzing the hydrolysis of coenzyme A (CoA) and various CoA esters, especially short- and medium-chain acyl-CoAs, to produce 3’,5’-ADP and 4’-phosphopantetheine derivatives. It prefers manganese and magnesium ions as cofactors and is implicated in the regulation of mitochondrial CoA and CoA ester homeostasis. The enzyme is involved in catabolic processes affecting nucleotide and coenzyme metabolism and is associated with metabolic pathways including the metabolism of water-soluble vitamins and cofactors. Mutations in or dysregulation of NUDT8 have been associated with rare hereditary metabolic conditions, but there are no therapeutics specifically targeting this enzyme at present.

Other names

Mitochondrial coenzyme A diphosphatase NUDT8Nudix motif 8FLJ41567Nucleoside diphosphate-linked moiety X motif 8, mitochondrialnucleoside diphosphate-linked moiety X motif 8, mitochondrial coenzyme A diphosphatase NUDT8nudix (nucleoside diphosphate linked moiety X)-type motif 8nudix motif 8