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

Stable Identifier
R-HSA-70785
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
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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
3082934 Deficiency of 3-methylglutaconyl-coenzyme A hydratase in two siblings with 3-methylglutaconic aciduria.

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

J Clin Invest 1986
12434311 3-Methylglutaconic aciduria type I is caused by mutations in AUH.

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

Am J Hum Genet 2002
11738050 Crystal structure of human AUH protein, a single-stranded RNA binding homolog of enoyl-CoA hydratase

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

Structure 2001
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
methylglutaconyl-CoA hydratase activity of AUH hexamer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea