ACSL4

ACSL4 is an isozyme of the 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

Full Name

Acyl-CoA Synthetase Long Chain Family Member 4

Function

Catalyzes the conversion of long-chain fatty acids to their active form acyl-CoA for both synthesis of cellular lipids, and degradation via beta-oxidation. Preferentially activates arachidonate and eicosapentaenoate as substrates. Preferentially activates 8,9-EET> 14,15-EET> 5,6-EET> 11,12-EET. Modulates glucose-stimulated insulin secretion by regulating the levels of unesterified EETs (By similarity). Modulates prostaglandin E2 secretion.

Biological Process

Dendritic spine development
Embryonic process involved in female pregnancy
Fatty acid metabolic process
Fatty acid transport
Lipid biosynthetic process
Lipid metabolic process
Long-chain fatty acid metabolic process
Long-chain fatty-acyl-CoA biosynthetic process
Long-chain fatty-acyl-CoA metabolic process
Negative regulation of prostaglandin secretion
Neuron differentiation
Positive regulation of cell growth
Positive regulation of insulin secretion
Response to interleukin-15
Response to nutrient
Triglyceride biosynthetic process

Cellular Location

Cell membrane; Microsome membrane; Endoplasmic reticulum membrane; Peroxisome membrane; Mitochondrion outer membrane

Involvement in disease

Mental retardation, X-linked 63 (MRX63): A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period. Intellectual deficiency is the only primary symptom of non-syndromic X-linked mental retardation, while syndromic mental retardation presents with associated physical, neurological and/or psychiatric manifestations.
Alport syndrome with mental retardation, midface hypoplasia and elliptocytosis (ATS-MR): An X-linked contiguous gene deletion syndrome characterized by glomerulonephritis, sensorineural hearing loss, mental retardation, midface hypoplasia and elliptocytosis.

Topology

Helical: 8-28 aa
Cytoplasmic: 29-711 aa