Names & Taxonomy

Uniprot ID:
P50416
Entry Name:
CPT1A_HUMAN
Status:
reviewed
Protein Names:
Carnitine O-palmitoyltransferase 1, liver isoform (CPT1-L) (EC 2.3.1.21) (Carnitine O-palmitoyltransferase I, liver isoform) (CPT I) (CPTI-L) (Carnitine palmitoyltransferase 1A)
Gene Names:
CPT1A CPT1
Gene Names Primary:
CPT1A
Organism:
Homo sapiens (Human)

Structure

Length:
773
Sequence:
MAEAHQAVAFQFTVTPDGIDLRLSHEALRQIYLSGLHSWKKKFIRFKNGIITGVYPASPSSWLIVVVGVMTTMYAKIDPSLGIIAKINRTLETANCMSSQTKNVVSGVLFGTGLWVALIVTMRYSLKVLLSYHGWMFTEHGKMSRATKIWMGMVKIFSGRKPMLYSFQTSLPRLPVPAVKDTVNRYLQSVRPLMKEEDFKRMTALAQDFAVGLGPRLQWYLKLKSWWATNYVSDWWEEYIYLRGRGPLMVNSNYYAMDLLYILPTHIQAARAGNAIHAILLYRRKLDREEIKPIRLLGSTIPLCSAQWERMFNTSRIPGEETDTIQHMRDSKHIVVYHRGRYFKVWLYHDGRLLKPREMEQQMQRILDNTSEPQPGEARLAALTAGDRVPWARCRQAYFGRGKNKQSLDAVEKAAFFVTLDETEEGYRSEDPDTSMDSYAKSLLHGRCYDRWFDKSFTFVVFKNGKMGLNAEHSWADAPIVAHLWEYVMSIDSLQLGYAEDGHCKGDINPNIPYPTRLQWDIPGECQEVIETSLNTANLLANDVDFHSFPFVAFGKGIIKKCRTSPDAFVQLALQLAHYKDMGKFCLTYEASMTRLFREGRTETVRSCTTESCDFVRAMVDPAQTVEQRLKLFKLASEKHQHMYRLAMTGSGIDRHLFCLYVVSKYLAVESPFLKEVLSEPWRLSTSQTPQQQVELFDLENNPEYVSSGGGFGPVADDGYGVSYILVGENLINFHISSKFSCPETDSHRFGRHLKEAMTDIITLFGLSSNSKK
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion outer membrane

Function

Function:
Catalyzes 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.
Pathway:
Lipid metabolism; fatty acid beta-oxidation.
Catalytic Activity:
Palmitoyl-CoA + L-carnitine = CoA + L-palmitoylcarnitine.
Enzyme Regulation:
ENZYME REGULATION: Inhibited by malonyl-CoA.
Active Site:
ACT_SITE 473 473 Proton acceptor.
Cross Reference Drug Bank:
DB01016 DB00583 DB01074
Gene Ontology Go:
integral component of mitochondrial outer membrane
membrane
mitochondrial inner membrane
mitochondrial outer membrane
mitochondrion
carnitine O-palmitoyltransferase activity
palmitoleoyltransferase activity
carnitine metabolic process
carnitine shuttle
cellular lipid metabolic process
cellular response to fatty acid
circadian rhythm
eating behavior
epithelial cell differentiation
fatty acid beta-oxidation
glucose metabolic process
long-chain fatty acid metabolic process
positive regulation of fatty acid beta-oxidation
protein homooligomerization
regulation of insulin secretion
response to drug
response to organic cyclic compound
small molecule metabolic process
triglyceride metabolic process
Gene Ontology Biological Process:
carnitine metabolic process
carnitine shuttle
cellular lipid metabolic process
cellular response to fatty acid
circadian rhythm
eating behavior
epithelial cell differentiation
fatty acid beta-oxidation
glucose metabolic process
long-chain fatty acid metabolic process
positive regulation of fatty acid beta-oxidation
protein homooligomerization
regulation of insulin secretion
response to drug
response to organic cyclic compound
small molecule metabolic process
triglyceride metabolic process
Gene Ontology Molecular Function:
carnitine O-palmitoyltransferase activity
palmitoleoyltransferase activity
Gene Ontology Cellular Component:
integral component of mitochondrial outer membrane
membrane
mitochondrial inner membrane
mitochondrial outer membrane
mitochondrion
Keywords:
3D-structure
Acetylation
Acyltransferase
Alternative splicing
Complete proteome
Disease mutation
Fatty acid metabolism
Lipid metabolism
Membrane
Mitochondrion
Mitochondrion outer membrane
Nitration
Phosphoprotein
Polymorphism
Reference proteome
Transferase
Transmembrane
Transmembrane helix
Transport

Publication

PubMed ID:
7892212 12189492 15489334 16271724 21269460 25944712 14711372 21990363 9691089 11441142 11350182 12111367 14517221 15669684 15110323