Methylglutaconyl-CoA hydratase, mitochondrial (AUH)

Methylglutaconyl-CoA hydratase, mitochondrial (AUH), is a bifunctional mitochondrial enzyme encoded by the AUH gene. It catalyzes a key step (the hydration of 3-methylglutaconyl-CoA to 3-hydroxy-3-methylglutaryl-CoA) in the catabolism of the amino acid leucine[1][4][5][7]. Uniquely, AUH can also bind AU-rich RNA elements found in the 3' untranslated regions of certain mRNAs, linking mitochondrial metabolism and post-transcriptional gene regulation[3][5]. Loss of AUH function leads to 3-methylglutaconic aciduria type I, a rare autosomal recessive disease characterized by the accumulation of organic acids and neurological problems due to defective mitochondrial leucine breakdown[4][7]. AUH is important for mitochondrial RNA metabolism, protein synthesis, and overall mitochondrial morphology and function[1][6]. There are no known drugs that directly target AUH, and it is not currently considered a direct therapeutic target in drug development, but it is important in the pathology of certain metabolic disorders[5][6].

Other names

3-Methylglutaconyl-CoA hydrataseMG-CoA hydrataseAU RNA binding methylglutaconyl-CoA hydrataseAU RNA-binding protein/enoyl-CoA hydrataseAU-binding protein/enoyl-CoA hydrataseAU-specific RNA-binding enoyl-CoA hydrataseItaconyl-CoA hydratase3-MG-CoA hydrataseAU RNA binding protein/enoyl-Coenzyme A hydratase