LIPID MAPS® Gene/Proteome Database (LMPD)

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LMPD Record

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LMP006785

UniProt Annotations

Entry Information
Gene Nameacyl-CoA synthetase family member 3
Protein EntryACSF3_HUMAN
UniProt IDQ4G176
SpeciesHuman
Comments
Comment typeDescription
DiseaseCombined malonic and methylmalonic aciduria (CMAMMA) [MIM
FunctionCatalyzes 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.
Sequence CautionSequence=AAH72391.1; Type=Miscellaneous discrepancy; Note=Aberrant splicing.; Evidence= ;
SimilarityBelongs to the ATP-dependent AMP-binding enzyme family.
Subcellular LocationMitochondrion .