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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
General
fatty-acid CoA ligase
mitochondrial protein
Properties
SIZE: entity length = 711 aa
MW = 79 kD
COMPARTMENT: mitochondria
peroxisome
endoplasmic reticulum
MOTIF: transmembrane domain {8-28}
Database Correlations
OMIM correlations
UniProt O60488
Pfam PF00501
Entrez Gene 2182
KEGG correlations
ENZYME 6.2.1.3
References
- UniProt :accession O60488
- GeneReviews
http://www.ncbi.nlm.nih.gov/sites/genetests/lab/gene/ACSL4