Very-long-chain (3R)-3-hydroxyacyl-CoA dehydratase 3 (HACD3)

Very-long-chain (3R)-3-hydroxyacyl-CoA dehydratase 3 (HACD3) is an endoplasmic reticulum-associated enzyme responsible for the dehydration of 3-hydroxyacyl-CoA to trans-2,3-enoyl-CoA, constituting the third step in the elongation cycle of very-long-chain fatty acids (VLCFAs). This process is vital for generating membrane lipids and lipid mediators of various chain lengths, impacting numerous physiological processes. HACD3 is also implicated in certain signaling pathways, notably Rac1 signaling and insulin receptor function. Mutations or dysregulation of HACD3 have been associated with disorders involving VLCFA metabolism, such as developmental and epileptic encephalopathy and Fiedler's myocarditis[1][2][6].

Other names

BIND1PTPLAD1B-ind1hB-ind1B-IND1HSPC121Butyrate-induced protein 1Butyrate-induced transcript 1Protein-tyrosine phosphatase-like A domain-containing protein 1Protein tyrosine phosphatase-like protein PTPLAD1Very-long-chain (3R)-3-hydroxyacyl-[acyl-carrier protein] dehydratase 3