Reactome | HSD17B4 hydrates trans-2,3-dehydrohexacosanoyl-CoA

HSD17B4 hydrates trans-2,3-dehydrohexacosanoyl-CoA

Stable Identifier

R-HSA-390252

Type

Reaction [transition]

Species

Homo sapiens

Compartment

peroxisomal matrix

Synonyms

trans-2,3-dehydrohexacosanoyl-CoA + H2O => 3-hydroxyhexacosanoyl-CoA

ReviewStatus

5/5

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HSD17B4 hydrates trans-2,3-dehydrohexacosanoyl-CoA

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Peroxisomal HSD17B4 dimer catalyzes the reaction of trans-2,3-dehydrohexacosanoyl-CoA and H2O to form 3-hydroxyhexacosanoyl-CoA. The enzyme is bifunctional - an aminoterminal domain catalyzes the dehydrogenation of a variety of 3-hydroxyacyl-CoA's and a carboxyterminal domain catalyzes the hydration of a variety of trans-2,3-dehydroacyl-CoA's, the reaction annotated here (Jiang et al. 1996, 1997). Defects in the enzyme are associated with a severe disorder of peroxisomal fatty acid metabolism in humans (Ferdinandusse et al. 2006).

Literature References

PubMed ID	Title	Journal	Year
9089413	Structure of D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein Souri, M, Hashimoto, T, Jiang, LL, Miyazawa, S	J Biochem	1997
16385454	Mutational spectrum of D-bifunctional protein deficiency and structure-based genotype-phenotype analysis Glumoff, T, Koski, MK, Oostheim, W, Wanders, RJA, Gloerich, J, Ylianttila, MS, Hiltunen, JK, Waterham, HR, Ferdinandusse, S	Am J Hum Genet	2006
8902629	Purification and properties of human D-3-hydroxyacyl-CoA dehydratase: medium-chain enoyl-CoA hydratase is D-3-hydroxyacyl-CoA dehydratase Fukushima, H, Kobayashi, A, Matsuura, H, Hashimoto, T, Jiang, LL	J Biochem (Tokyo)	1996