long-chain-fatty-acid--CoA ligase 6; long-chain acyl-CoA synthetase 6; LACS 6 (ACSL6, ACS2, FACL6, KIAA0837, LACS5)

Function

• activation of long-chain fatty acids for both synthesis of cellular lipids, & degradation via beta-oxidation
• role in fatty acid metabolism in brain
• the acyl-CoAs produced may be utilized exclusively for synthesis of brain lipid

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

Cofactor: Mg+2

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=8

Expression

• expressed predominantly in erythrocyte precursors, in particular in reticulocytes, fetal blood cells derived from fetal liver, hemopoietic stem cells from cord blood, bone marrow & brain
• expression is low at earlier stages of erythroid development but is very high in reticulocytes

Pathology

• chromosomal translocation t(5;12)(q31;p13) involving ACSL6 with ETV6 may be a cause of
  • myelodysplastic syndrome with basophilia
  • acute myelogenous leukemia with eosinophilia
  • acute eosinophilic leukemia

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=23305
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:23305
• OMIM: https://mirror.omim.org/entry/604443
• UniProt: http://www.uniprot.org/uniprot/Q9UKU0.html