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

KEGG Pathway ID Description
mmu04152 AMPK signaling pathway
mmu04920 Adipocytokine signaling pathway
mmu03320 PPAR signaling pathway

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]