Acyl-coenzyme A diphosphatase NUDT19 (NUDT19)

Acyl-coenzyme A diphosphatase NUDT19 (NUDT19) is a peroxisome-localized hydrolase that specifically hydrolyzes coenzyme A (CoA) and acyl-CoA esters, yielding 3′,5′-ADP and 4′-(acyl)phosphopantetheine. NUDT19 regulates the local CoA pool in the kidney, playing a distinct metabolic role compared to its liver counterpart, Nudt7. Its enzymatic activity is crucial for peroxisomal lipid metabolic pathways, including the oxidation of dicarboxylic and very long-chain fatty acids. NUDT19 is encoded by the NUDT19 gene and is classified within the Nudix hydrolase family of enzymes. Inhibition of NUDT19, such as by chenodeoxycholic acid, can elevate kidney CoA levels and disrupt metabolic balance. Disease association studies implicate possible roles in neurodegeneration with brain iron accumulation, but NUDT19 is not recognized as a major direct therapeutic target for approved drugs.

Other names

Nudix hydrolase 19Nudix motif 19RP2CTC-379B2.4nucleoside diphosphate-linked moiety X motif 19

Competitive inhibition of CoA binding/hydrolysis (e.g., by chenodeoxycholic acid)