catalytic activity:(24R,25R)-3-alpha,7-alpha,12-alpha,24-tetrahydroxy-5-beta-cholestanoyl-CoA = (24E)-3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholest-24-enoyl-CoA + H(2)O.,catalytic activity:(S)-3-hydroxyacyl-CoA + NAD(+) = 3-oxoacyl-CoA + NADH.,disease:Defects in HSD17B4 are a cause of D-bifunctional protein deficiency (DBPD) [MIM:261515]. DBPD is a disorder of peroxisomal fatty acid beta-oxidation.,function:Bifunctional enzyme acting on the peroxisomal beta-oxidation pathway for fatty acids. Catalyzes the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids.,pathway:Lipid metabolism; fatty acid beta-oxidation.,similarity:Belongs to the short-chain dehydrogenases/reductases (SDR) family.,similarity:Contains 1 SCP2 domain.,tissue specificity:Present in many tissues with highest concentrations in liver, heart, prostate and testis.,
Peroxisome .
Present in many tissues with highest concentrations in liver, heart, prostate and testis.
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