由該基因編碼的蛋白質是參與脂肪酸過氧化物酶體β氧化途徑的雙功能酶。它還可以催化直鏈和2-甲基支鏈脂肪酸形成3-酮酰基-CoA中間體。影響過氧化物酶體脂肪酸β氧化活性的基因缺陷是D-雙功能蛋白缺乏癥（DPD）的一個原因。這個基因的一個明顯的假基因存在于8號染色體上。已發現該基因的多個選擇性剪接轉錄變體編碼不同的亞型。[由RefSeq提供，2014年5月]
The protein encoded by this gene is a bifunctional enzyme that is involved in the peroxisomal beta-oxidation pathway for fatty acids. It also acts as a catalyst for the formation of 3-ketoacyl-CoA intermediates from both straight-chain and 2-methyl-branched-chain fatty acids. Defects in this gene that affect the peroxisomal fatty acid beta-oxidation activity are a cause of D-bifunctional protein deficiency (DBPD). An apparent pseudogene of this gene is present on chromosome 8. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, May 2014]