beta-methylglutaconyl-CoA + H2O <=> beta-hydroxy-beta-methylglutaryl-CoA

Stable Identifier
Reaction [transition]
Homo sapiens
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Mitochondrial ethylglutaconyl-CoA hydratase (AUH) catalyzes the hydrolysis of beta-methylglutaconyl-CoA to yield beta-hydroxy-beta-methylglutaryl-CoA (IJlst et al. 2002; Narisawa et al. 1986). Crystallographic studies have shown the active enzyme to be a hexamer of AUH polypeptides whose aminoterminal 67 residues, a mitochondrial targeting sequence, have been removed ((Kurimoto et al. 2001).

Literature References
PubMed ID Title Journal Year
12434311 3-Methylglutaconic aciduria type I is caused by mutations in AUH.

Duran, M, Lehnert, W, IJlst, L, Loupatty, FJ, Ruiter, JP

Am J Hum Genet 2002
3082934 Deficiency of 3-methylglutaconyl-coenzyme A hydratase in two siblings with 3-methylglutaconic aciduria.

Duran, M, Wadman, SK, Narisawa, K, Gibson, KM, Nyhan, WL, Sweetman, L

J Clin Invest 1986
11738050 Crystal structure of human AUH protein, a single-stranded RNA binding homolog of enoyl-CoA hydratase

Kurimoto, K, Yokoyama, S, Muto, Y, Nureki, O, Fukai, S

Structure 2001
Catalyst Activity

methylglutaconyl-CoA hydratase activity of AUH hexamer [mitochondrial matrix]

Orthologous Events
Cross References
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