LMPD Database

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LMP002018

UniProt Annotations

Entry Information
Gene Namecarnitine palmitoyltransferase 1A (liver)
Protein EntryCPT1A_HUMAN
UniProt IDQ8WZ48
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=P50416-1; Sequence=Displayed; Name=2; IsoId=P50416-2; Sequence=VSP_012167;
Catalytic ActivityPalmitoyl-CoA + L-carnitine = CoA + L- palmitoylcarnitine. {ECO
DiseaseCarnitine palmitoyltransferase 1A deficiency (CPT1AD) [MIM
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.
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.
InductionUp-regulated by fatty acids.
PathwayLipid metabolism; fatty acid beta-oxidation.
SimilarityBelongs to the carnitine/choline acetyltransferase family.
Subcellular LocationMitochondrion outer membrane ; Multi- pass membrane protein {ECO
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).
Tissue SpecificityStrong expression in kidney and heart, and lower in liver and skeletal muscle.