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

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

Stable Identifier

R-HSA-70785

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

3-methyl-(2E)-glutaconyl-CoA + H2O => (3S)-hydroxy-3-methylglutaryl-CoA

ReviewStatus

5/5

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

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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 amino-terminal 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