Reaction: 3-hydroxypristanoyl-CoA + NAD+ => 3-ketoxypristanoyl-CoA + NADH + H+
- in pathway: Beta-oxidation of pristanoyl-CoA
Peroxisomal HSD17B4 dimer catalyzes the reaction of 3-hydroxypristanoyl-CoA and NAD+ to form 3-ketoxypristanoyl-CoA and NADH + H+. The enzyme is bifunctional - an aminoterminal domain catalyzes the dehydrogenation of a variety of 3-hydroxyacyl-CoA's, the reaction annotated here, and a carboxyterminal domain catalyzes the hydration of a variety of trans-2,3-dehydroacyl-CoA's (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:
H+ [peroxisomal matrix]
3-ketopristanoyl-CoA [peroxisomal matrix]
NADH [peroxisomal matrix]
3-hydroxypristanoyl-CoA [peroxisomal matrix]
NAD+ [peroxisomal matrix]
Reactome.org reaction link: R-HSA-389995
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Reaction input - small molecules:
3-hydroxypristanoyl-CoA
NAD(1-)
Reaction output - small molecules:
hydron
3-oxopristanoyl-CoA
NADH(2-)
Reactome.org link: R-HSA-389995