HSD17B4: Proteins and Enzymes

The protein encoded by the HSD17B4 gene is a bifunctional enzyme that is involved in the peroxisomal beta-oxidation pathwayfor fatty acids. It also acts as a catalyst for the formation of 3-ketoacyl-CoA intermediates from both straight-chainand 2-methyl-branched-chain fatty acids. Defects in this gene that affect the peroxisomal fatty acid beta-oxidationactivity are a cause of D-bifunctional protein deficiency (DBPD). An apparent pseudogene of this gene is present onchromosome 8. (provided by RefSeq)