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