Gene/Proteome Database (LMPD)
LMPD ID
LMP000118
Gene ID
Species
Mus musculus (Mouse)
Gene Name
carnitine palmitoyltransferase 1a, liver
Gene Symbol
Synonyms
C730027G07; CPTI; Cpt1
Alternate Names
carnitine O-palmitoyltransferase 1, liver isoform; CPT I; CPT1-L; CPTI-L; L-CPT I; carnitine palmitoyltransferase 1, liver; carnitine O-palmitoyltransferase I, liver isoform
Chromosome
19
Map Location
19 A|19 3.08 cM
EC Number
2.3.1.21
Proteins
carnitine O-palmitoyltransferase 1, liver isoform | |
---|---|
Refseq ID | NP_038523 |
Protein GI | 162287142 |
UniProt ID | P97742 |
mRNA ID | NM_013495 |
Length | 773 |
RefSeq Status | VALIDATED |
MAEAHQAVAFQFTVTPDGIDLRLSHEALKQICLSGLHSWKKKFIRFKNGIITGVFPASPSSWLIVVVGVISSMHTKVDPSLGMIAKINRTLDTTGRMSSQTKNIVSGVLFGTGLWVAIIMTMRYSLKVLLSYHGWMFAEHGKMSRSTRIWMAMVKVFSGRKPMLYSFQTSLPRLPVPAVKDTVSRYLESVRPLMKEGDFQRMTALAQDFAVNLGPKLQWYLKLKSWWATNYVSDWWEEYIYLRGRGPIMVNSNYYAMEMLYITPTHIQAARAGNTIHAILLYRRTVDREELKPIRLLGSTIPLCSAQWERLFNTSRIPGEETDTIQHVKDSRHIVVYHRGRYFKVWLYHDGRLLRPRELEQQMQQILDDTSEPQPGEAKLAALTAADRVPWAKCRQTYFARGKNKQSLDAVEKAAFFVTLDESEQGYREEDPEASIDSYAKSLLHGRCFDRWFDKSITFVVFKNSKIGINAEHSWADAPIVGHLWEYVMATDVFQLGYSEDGHCKGDKNPNIPKPTRLQWDIPGECQEVIETSLSSASFLANDVDLHSFPFDTFGKGLIKKCRTSPDAFIQLALQLAHYKDMGKFCLTYEASMTRLFREGRTETVRSCTTESCNFVLAMMDPTTTAEQRFKLFKIACEKHQHLYRLAMTGAGIDRHLFCLYVVSKYLAVDSPFLKEVLSEPWRLSTSQTPQQQVELFDFEKYPDYVSCGGGFGPVADDGYGVSYIIVGENFIHFHISSKFSSPETDSHRFGKHLRQAMMDIITLFGLTANSKK |
Gene Information
Entrez Gene ID
Gene Name
carnitine palmitoyltransferase 1a, liver
Gene Symbol
Species
Mus musculus
Gene Ontology (GO Annotations)
GO ID | Source | Type | Description |
---|---|---|---|
GO:0031307 | ISS:UniProtKB | C | integral component of mitochondrial outer membrane |
GO:0005743 | IDA:MGI | C | mitochondrial inner membrane |
GO:0005739 | IDA:MGI | C | mitochondrion |
GO:0004095 | ISS:UniProtKB | F | carnitine O-palmitoyltransferase activity |
GO:0009437 | ISS:UniProtKB | P | carnitine metabolic process |
GO:0071398 | IEA:Ensembl | P | cellular response to fatty acid |
GO:0042755 | IEA:Ensembl | P | eating behavior |
GO:0030855 | IEA:Ensembl | P | epithelial cell differentiation |
GO:0006635 | IEA:UniProtKB-UniPathway | P | fatty acid beta-oxidation |
GO:0006006 | IEA:Ensembl | P | glucose metabolic process |
GO:0001676 | ISS:UniProtKB | P | long-chain fatty acid metabolic process |
GO:0032000 | IEA:Ensembl | P | positive regulation of fatty acid beta-oxidation |
GO:0051260 | IEA:Ensembl | P | protein homooligomerization |
GO:0050796 | IEA:Ensembl | P | regulation of insulin secretion |
GO:0042493 | IEA:Ensembl | P | response to drug |
GO:0014070 | IEA:Ensembl | P | response to organic cyclic compound |
GO:0006810 | IEA:UniProtKB-KW | P | transport |
GO:0006641 | IEA:Ensembl | P | triglyceride metabolic process |
KEGG Pathway Links
Domain Information
InterPro Annotations
Accession | Description |
---|---|
IPR000542 | Acyltransferase ChoActase/COT/CPT |
UniProt Annotations
Entry Information
Gene Name
carnitine palmitoyltransferase 1a, liver
Protein Entry
CPT1A_MOUSE
UniProt ID
Species
Mouse
Comments
Comment Type | Description |
---|---|
Catalytic Activity | Palmitoyl-CoA + L-carnitine = CoA + L- palmitoylcarnitine. {ECO:0000269|PubMed:16169268}. |
Disruption Phenotype | Complete 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}. |
Domain | A 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 Regulation | Inhibited by malonyl-CoA. |
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. {ECO:0000269|PubMed:16169268, ECO:0000269|PubMed:17030509}. |
Pathway | Lipid metabolism; fatty acid beta-oxidation. |
Similarity | Belongs to the carnitine/choline acetyltransferase family. {ECO:0000305}. |
Subcellular Location | Mitochondrion outer membrane; Multi-pass membrane protein. |
Subunit | Homohexamer 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}. |
Identical and Related Proteins
Unique RefSeq proteins for LMP000118 (as displayed in Record Overview)
Protein GI | Database | Accession | Length | Protein Name |
---|---|---|---|---|
162287142 | RefSeq | NP_038523 | 773 | carnitine O-palmitoyltransferase 1, liver isoform |
Identical Sequences to LMP000118 proteins
Reference | Database | Accession | Length | Protein Name |
---|---|---|---|---|
GI:162287142 | RefSeq | XP_006531717.1 | 773 | PREDICTED: carnitine O-palmitoyltransferase 1, liver isoform isoform X1 [Mus musculus] |
GI:162287142 | RefSeq | XP_006531718.1 | 773 | PREDICTED: carnitine O-palmitoyltransferase 1, liver isoform isoform X2 [Mus musculus] |
GI:162287142 | RefSeq | XP_006531719.1 | 773 | PREDICTED: carnitine O-palmitoyltransferase 1, liver isoform isoform X3 [Mus musculus] |
GI:162287142 | RefSeq | XP_006531720.1 | 773 | PREDICTED: carnitine O-palmitoyltransferase 1, liver isoform isoform X4 [Mus musculus] |
GI:162287142 | RefSeq | XP_006531721.1 | 773 | PREDICTED: carnitine O-palmitoyltransferase 1, liver isoform isoform X5 [Mus musculus] |
GI:162287142 | RefSeq | XP_006531722.1 | 773 | PREDICTED: carnitine O-palmitoyltransferase 1, liver isoform isoform X6 [Mus musculus] |
Related Sequences to LMP000118 proteins
Reference | Database | Accession | Length | Protein Name |
---|---|---|---|---|
GI:162287142 | DBBJ | BAC36808.1 | 773 | unnamed protein product [Mus musculus] |
GI:162287142 | DBBJ | BAE28126.1 | 773 | unnamed protein product [Mus musculus] |
GI:162287142 | DBBJ | BAF79672.1 | 773 | acyltransferase [Mus musculus] |
GI:162287142 | GenBank | AAH38395.1 | 773 | Carnitine palmitoyltransferase 1a, liver [Mus musculus] |
GI:162287142 | GenBank | AAH46383.1 | 773 | Carnitine palmitoyltransferase 1a, liver [Mus musculus] |
GI:162287142 | GenBank | EDL32932.1 | 786 | carnitine palmitoyltransferase 1a, liver, isoform CRA_b, partial [Mus musculus] |