HSD17B4 : hydroxysteroid 17-beta dehydrogenase 4
Description
The HSD17B4 (hydroxysteroid 17-beta dehydrogenase 4) is a protein-coding gene located on chromosome 5.
The HSD17B4 gene encodes the D-bifunctional protein, an enzyme located in peroxisomes that plays a crucial role in the breakdown of fatty acids. It has two separate domains: the hydratase and dehydrogenase domains, which catalyze the second and third steps, respectively, of the peroxisomal fatty acid beta-oxidation pathway. This pathway shortens fatty acids by two carbon atoms at a time until they are converted into acetyl-CoA, which is then used by the cell.
HSD17B4 is a bifunctional enzyme involved in the peroxisomal fatty acid beta-oxidation pathway. It catalyzes two key steps in fatty acid degradation: hydration of 2-enoyl-CoA (trans-2-enoyl-CoA) to produce (3R)-3-hydroxyacyl-CoA, and dehydrogenation of (3R)-3-hydroxyacyl-CoA to produce 3-ketoacyl-CoA (3-oxoacyl-CoA). This 3-ketoacyl-CoA is then further processed by SCPx. HSD17B4 can utilize various substrates, including straight-chain and branched-chain fatty acids, as well as bile acid intermediates.
HSD17B4 is also known as DBP, MFE-2, MFP-2, MPF-2, PRLTS1, SDR8C1.
