Malonate--CoA ligase ACSF3, mitochondrial

Definition:

Catalyzes the initial reaction in intramitochondrial fatty acid synthesis, by activating malonate and methylmalonate, but not acetate, into their respective CoA thioester. May have some preference toward very-long-chain substrates.

References:

[1]. Paul A Watkins, et al. Evidence for 26 distinct acyl-coenzyme A synthetase genes in the human genome. J Lipid Res. 2007 Dec;48(12):2736-50. [Content Brief]

[2]. Jennifer L Sloan, et al. Exome sequencing identifies ACSF3 as a cause of combined malonic and methylmalonic aciduria. Nat Genet. 2011 Aug 14;43(9):883-6. [Content Brief]

[3]. Andrzej Witkowski, et al. Mammalian ACSF3 protein is a malonyl-CoA synthetase that supplies the chain extender units for mitochondrial fatty acid synthesis. J Biol Chem. 2011 Sep 30;286(39):33729-36. [Content Brief]

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