long-chain-fatty-acid-CoA ligase 4; long-chain acyl-CoA synthetase 4; LACS 4 (ACSL4, ACS4, FACL4, LACS4)

Function

• activation of long-chain fatty acids for both synthesis of cellular lipids, & degradation via beta-oxidation
• preferentially uses arachidonate & eicosapentaenoate as substrates

ATP + a long-chain carboxylic acid + CoA = AMP + diphosphate + an acyl-CoA

Cofactor: Mg+2 (putative)

Structure

• belongs to the ATP-dependent AMP-binding enzyme family

Compartment

• mitochondrial outer membrane
• peroxisome membrane
• microsome membrane
• endoplasmic reticulum membrane

single-pass type 3 membrane protein (putative)

Alternative splicing

named isoforms=2; long, short

Pathology

• defects in ACSL4 are the cause of mental retardation X-linked type 63
• defects in ACSL4 are involved in Alport syndrome with mental retardation midface hypoplasia

More general terms

• fatty-acid CoA ligase
• mitochondrial protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=2182
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:2182
• OMIM: https://mirror.omim.org/entry/300157
• OMIM: https://mirror.omim.org/entry/300194
• OMIM: https://mirror.omim.org/entry/300387
• UniProt: http://www.uniprot.org/uniprot/O60488.html