Reaction: HSD17B4 hydrates trans-2,3-dehydrohexacosanoyl-CoA
- in pathway: Beta-oxidation of very long chain fatty acids
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).
Reaction - small molecule participants:
3-hydroxyhexacosanoyl-CoA [peroxisomal matrix]
H2O [peroxisomal matrix]
trans-2,3-dehydrohexacosanoyl-CoA [peroxisomal matrix]
Reactome.org reaction link: R-HSA-390252
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Reaction input - small molecules:
water
trans-2-hexacosenoyl-CoA
Reaction output - small molecules:
3-hydroxyhexacosanoyl-CoA
Reactome.org link: R-HSA-390252