The protein encoded by this gene is an isozyme of long-chain fatty-acid-coenzyme A ligase family. Although differing in substrate specificity, subcellular localization, and tissue distribution, all isozymes of this family convert free long-chain fatty acids into fatty acyl-CoA esters, and thereby play a key role in lipid biosynthesis and fatty acid degradation. This isozyme activates long-chain, branched-chain and very-long-chain fatty acids containing 22 or more carbons to their CoA derivatives. It is expressed primarily in liver and kidney, and is present in both endoplasmic reticulum and peroxisomes but not in mitochondria. Its decreased peroxisomal enzyme activity is in part responsible for the biochemical pathology in X-linked adrenoleukodystrophy.
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Protein Aliases: ACSVL1; Arachidonate--CoA ligase; FACVL1; FATP-2; FATP2; Fatty acid transport protein 2; Fatty-acid-coenzyme A ligase, very long-chain 1; hFACVL1; HsT17226; Long-chain-fatty-acid--CoA ligase; Phytanate--CoA ligase; solute carrier family 27 (fatty acid transporter), member 2; Solute carrier family 27 member 2; THCA-CoA ligase; Very long-chain acyl-CoA synthetase; very long-chain fatty-acid-coenzyme A ligase 1; Very long-chain-fatty-acid-CoA ligase; very-long-chain acyl-CoA synthetase; VLACS; VLCS
Gene Aliases: ACSVL1; FACVL1; FATP2; hFACVL1; HsT17226; SLC27A2; VLACS; VLCS
UniProt ID: (Human) O14975
Entrez Gene ID: (Human) 11001
Molecular Function: ligase