LMPD Database

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LMP000118

UniProt Annotations

Entry Information
Gene Namecarnitine palmitoyltransferase 1a, liver
Protein EntryCPT1A_MOUSE
UniProt IDP97742
SpeciesMouse
Comments
Comment typeDescription
Catalytic ActivityPalmitoyl-CoA + L-carnitine = CoA + L- palmitoylcarnitine. {ECO:0000269|PubMed:16169268}.
Disruption PhenotypeComplete lethality during early embryogenesis. Heterozygous mice are prone to liver steatosis. Compared to wild-type, heterozygotes show minor differences in blood glucose levels and in serum free fatty acid levels after fasting. {ECO:0000269|PubMed:16169268, ECO:0000269|PubMed:22229081}.
DomainA conformation change in the N-terminal region spanning the first 42 residues plays an important role in the regulation of enzyme activity by malonyl-CoA. {ECO:0000250}.
Enzyme RegulationInhibited by malonyl-CoA.
FunctionCatalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Plays an important role in triglyceride metabolism. {ECO:0000269|PubMed:16169268, ECO:0000269|PubMed:17030509}.
PathwayLipid metabolism; fatty acid beta-oxidation.
SimilarityBelongs to the carnitine/choline acetyltransferase family. {ECO:0000305}.
Subcellular LocationMitochondrion outer membrane; Multi-pass membrane protein.
SubunitHomohexamer and homotrimer. Identified in a complex that contains at least CPT1A, ACSL1 and VDAC1. Also identified in complexes with ACSL1 and VDAC2 and VDAC3 (By similarity). {ECO:0000250}.