Cpt1a

Functionⁱ

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.2 Publications

Catalytic activityⁱ

Palmitoyl-CoA + L-carnitine = CoA + L-palmitoylcarnitine.1 Publication

Activity regulationⁱ

Inhibited by malonyl-CoA.

Pathwayⁱ: fatty acid beta-oxidation

This protein is involved in the pathway fatty acid beta-oxidation, which is part of Lipid metabolism.
View all proteins of this organism that are known to be involved in the pathway fatty acid beta-oxidation and in Lipid metabolism.

Sites

Feature key	Position(s)	DescriptionActions	Length
Active siteⁱ	473	Proton acceptorBy similarity	1
Binding siteⁱ	589	CarnitineBy similarity	1
Binding siteⁱ	602	CarnitineBy similarity	1

GO - Molecular functionⁱ

carnitine O-palmitoyltransferase activity Source: UniProtKB
identical protein binding Source: MGI
palmitoleoyltransferase activity
Source: UniProtKB
Inferred from direct assayⁱ
- 12920182

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

carnitine metabolic process Source: UniProtKB
cellular response to fatty acid Source: Ensembl
eating behavior Source: MGI
epithelial cell differentiation Source: Ensembl
fatty acid beta-oxidation Source: UniProtKB-UniPathway
glucose metabolic process Source: MGI
long-chain fatty acid metabolic process Source: UniProtKB
positive regulation of fatty acid beta-oxidation Source: MGI
protein homooligomerization Source: MGI
regulation of fatty acid oxidation Source: MGI
regulation of insulin secretion Source: MGI
response to drug Source: Ensembl
response to organic cyclic compound Source: Ensembl
triglyceride metabolic process Source: MGI

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Acyltransferase, Transferase
Biological process	Fatty acid metabolism, Lipid metabolism, Transport

Enzyme and pathway databases

Reactomeⁱ	R-MMU-200425 Import of palmitoyl-CoA into the mitochondrial matrix R-MMU-5362517 Signaling by Retinoic Acid
UniPathwayⁱ	UPA00659

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: Carnitine O-palmitoyltransferase 1, liver isoform (EC:2.3.1.21) Short name: CPT1-L Alternative name(s): Carnitine O-palmitoyltransferase I, liver isoform Short name: CPT I Short name: CPTI-L Carnitine palmitoyltransferase 1A
Gene namesⁱ	Name:Cpt1a Synonyms:Cpt-1, Cpt1
Organismⁱ	Mus musculus (Mouse)
Taxonomic identifierⁱ	10090 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Mus › Mus
Proteomesⁱ	UP000000589 Componentⁱ: Chromosome 19

Organism-specific databases

MGIⁱ	MGI:1098296 Cpt1a

Topology

Feature key	Position(s)	DescriptionActions	Length
Topological domainⁱ	2 – 47	CytoplasmicSequence analysisAdd BLAST	46
Transmembraneⁱ	48 – 73	HelicalSequence analysisAdd BLAST	26
Topological domainⁱ	74 – 102	Mitochondrial intermembraneSequence analysisAdd BLAST	29
Transmembraneⁱ	103 – 122	HelicalSequence analysisAdd BLAST	20
Topological domainⁱ	123 – 773	CytoplasmicSequence analysisAdd BLAST	651

Keywords - Cellular componentⁱ

Membrane, Mitochondrion, Mitochondrion outer membrane

Pathology & Biotechⁱ

Disruption phenotypeⁱ

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.2 Publications

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Initiator methionineⁱ		RemovedBy similarity
ChainⁱPRO_0000210160	2 – 773	Carnitine O-palmitoyltransferase 1, liver isoformAdd BLAST		772

Amino acid modifications

Feature key	Position(s)	DescriptionActions	Length
Modified residueⁱ	2	N-acetylalanineBy similarity	1
Modified residueⁱ	282	Nitrated tyrosineBy similarity	1
Modified residueⁱ	588	PhosphothreonineBy similarity	1
Modified residueⁱ	589	Nitrated tyrosineBy similarity	1
Modified residueⁱ	604	PhosphothreonineBy similarity	1
Modified residueⁱ	741	PhosphoserineBy similarity	1
Modified residueⁱ	747	PhosphoserineBy similarity	1

Keywords - PTMⁱ

Acetylation, Nitration, Phosphoprotein

Proteomic databases

EPDⁱ	P97742
PaxDbⁱ	P97742
PeptideAtlas More...PeptideAtlasⁱ	P97742
PRIDEⁱ	P97742

PTM databases

iPTMnetⁱ	P97742
PhosphoSitePlusⁱ	P97742
SwissPalmⁱ	P97742

Expressionⁱ

Gene expression databases

Bgeeⁱ	ENSMUSG00000024900 Expressed in 329 organ(s), highest expression level in adrenal gland
CleanExⁱ	MM_CPT1A
ExpressionAtlasⁱ	P97742 baseline and differential
Genevisibleⁱ	P97742 MM

Interactionⁱ

Subunit structureⁱ

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).By similarity

GO - Molecular functionⁱ

identical protein binding Source: MGI

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

BioGridⁱ	198863, 2 interactors
IntActⁱ	P97742, 1 interactor
STRINGⁱ	10090.ENSMUSP00000025835

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	P97742
SMRⁱ	P97742
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Region

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Regionⁱ	555 – 567	Coenzyme A bindingBy similarityAdd BLAST		13

Domainⁱ

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.By similarity

Sequence similaritiesⁱ

Belongs to the carnitine/choline acetyltransferase family.Curated

Keywords - Domainⁱ

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGⁱ	KOG3717 Eukaryota ENOG410XNZ9 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00760000119220
HOGENOMⁱ	HOG000233542
HOVERGENⁱ	HBG003458
InParanoidⁱ	P97742
KEGG Orthology (KO) More...KOⁱ	K08765
OMAⁱ	ASHMWEN
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G026C
PhylomeDBⁱ	P97742
TreeFamⁱ	TF313836

Family and domain databases

InterProⁱ	View protein in InterPro IPR000542 Carn_acyl_trans IPR039551 Cho/carn_acyl_trans IPR032476 CPT_N
PANTHERⁱ	PTHR22589 PTHR22589, 1 hit
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF00755 Carn_acyltransf, 1 hit PF16484 CPT_N, 1 hit
PROSITEⁱ	View protein in PROSITE PS00439 ACYLTRANSF_C_1, 1 hit PS00440 ACYLTRANSF_C_2, 1 hit

Sequenceⁱ

Sequence statusⁱ: Complete.

Sequence processingⁱ: The displayed sequence is further processed into a mature form.

P97742-1 [UniParc]FASTA Add to basket

« Hide

        10         20         30         40         50
MAEAHQAVAF QFTVTPDGID LRLSHEALKQ ICLSGLHSWK KKFIRFKNGI 
        60         70         80         90        100
ITGVFPASPS SWLIVVVGVI SSMHTKVDPS LGMIAKINRT LDTTGRMSSQ 
       110        120        130        140        150
TKNIVSGVLF GTGLWVAIIM TMRYSLKVLL SYHGWMFAEH GKMSRSTRIW 
       160        170        180        190        200
MAMVKVFSGR KPMLYSFQTS LPRLPVPAVK DTVSRYLESV RPLMKEGDFQ 
       210        220        230        240        250
RMTALAQDFA VNLGPKLQWY LKLKSWWATN YVSDWWEEYI YLRGRGPIMV 
       260        270        280        290        300
NSNYYAMEML YITPTHIQAA RAGNTIHAIL LYRRTVDREE LKPIRLLGST 
       310        320        330        340        350
IPLCSAQWER LFNTSRIPGE ETDTIQHVKD SRHIVVYHRG RYFKVWLYHD 
       360        370        380        390        400
GRLLRPRELE QQMQQILDDT SEPQPGEAKL AALTAADRVP WAKCRQTYFA 
       410        420        430        440        450
RGKNKQSLDA VEKAAFFVTL DESEQGYREE DPEASIDSYA KSLLHGRCFD 
       460        470        480        490        500
RWFDKSITFV VFKNSKIGIN AEHSWADAPI VGHLWEYVMA TDVFQLGYSE 
       510        520        530        540        550
DGHCKGDKNP NIPKPTRLQW DIPGECQEVI ETSLSSASFL ANDVDLHSFP 
       560        570        580        590        600
FDTFGKGLIK KCRTSPDAFI QLALQLAHYK DMGKFCLTYE ASMTRLFREG 
       610        620        630        640        650
RTETVRSCTT ESCNFVLAMM DPTTTAEQRF KLFKIACEKH QHLYRLAMTG 
       660        670        680        690        700
AGIDRHLFCL YVVSKYLAVD SPFLKEVLSE PWRLSTSQTP QQQVELFDFE 
       710        720        730        740        750
KYPDYVSCGG GFGPVADDGY GVSYIIVGEN FIHFHISSKF SSPETDSHRF 
       760        770 
GKHLRQAMMD IITLFGLTAN SKK

Length:773

Mass (Da):88,251

Last modified:July 5, 2004 - v4

Checksum:ⁱ85C3D386FFD97C5E

GO

Experimental Info

Feature key	Position(s)	DescriptionActions	Length
Sequence conflictⁱ	145	R → C in AAH38395 (PubMed:15489334).Curated	1
Sequence conflictⁱ	145	R → C in AAH46383 (PubMed:15489334).Curated	1
Sequence conflictⁱ	398	Y → C in AAC31641 (PubMed:9680378).Curated	1
Sequence conflictⁱ	531	E → D in BAC36808 (PubMed:16141072).Curated	1
Sequence conflictⁱ	630	F → L in AAC31641 (PubMed:9680378).Curated	1
Sequence conflictⁱ	732	I → T in AAC31641 (PubMed:9680378).Curated	1
Sequence conflictⁱ	760	D → A in AAC31641 (PubMed:9680378).Curated	1

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AK050213 mRNA Translation: BAC34126.2 AK077454 mRNA Translation: BAC36808.1 BC038395 mRNA Translation: AAH38395.1 BC046383 mRNA Translation: AAH46383.1 AF017175 mRNA Translation: AAC31641.1 S82796 mRNA Translation: AAB39370.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS29395.1
NCBI Reference Sequences More...RefSeqⁱ	NP_038523.2, NM_013495.2 XP_006531717.1, XM_006531654.2 XP_006531718.1, XM_006531655.2 XP_006531721.1, XM_006531658.3 XP_006531722.1, XM_006531659.2
UniGeneⁱ	Mm.18522

Genome annotation databases

Ensemblⁱ	ENSMUST00000025835; ENSMUSP00000025835; ENSMUSG00000024900
GeneIDⁱ	12894
KEGGⁱ	mmu:12894
UCSC genome browser More...UCSCⁱ	uc008fwf.2 mouse

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
P97742	Carnitine palmitoyltransferase 1a, liver		MOUSE		773	UniRef100_P97742
UPI0005ABB16C		MOUSE		798

Protein	Similar proteins		Species	Score	Length	Source
P97742	Carnitine O-palmitoyltransferase 1, liver isoform		RAT		773	UniRef90_P32198
Carnitine palmitoyltransferase 1a, liver		MOUSE		773
carnitine O-palmitoyltransferase 1, liver isoform-like		MESAU		148
UPI00038BCE1E		MICOH		773
UPI00042AC3BA		PERMB		773
+12

Protein	Similar proteins		Species	Score	Length	Source
P97742	Carnitine O-palmitoyltransferase 1, liver isoform		RAT		773	UniRef50_P32198
Carnitine O-palmitoyltransferase 1, liver isoform	)	HUMAN		773
Carnitine O-palmitoyltransferase 1, muscle isoform		BOVIN		771
Carnitine O-palmitoyltransferase 1, muscle isoform		PIG		772
Carnitine O-palmitoyltransferase 1, muscle isoform		MOUSE		772
+780

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AK050213 mRNA Translation: BAC34126.2 AK077454 mRNA Translation: BAC36808.1 BC038395 mRNA Translation: AAH38395.1 BC046383 mRNA Translation: AAH46383.1 AF017175 mRNA Translation: AAC31641.1 S82796 mRNA Translation: AAB39370.1
CCDSⁱ	CCDS29395.1
RefSeqⁱ	NP_038523.2, NM_013495.2 XP_006531717.1, XM_006531654.2 XP_006531718.1, XM_006531655.2 XP_006531721.1, XM_006531658.3 XP_006531722.1, XM_006531659.2
UniGeneⁱ	Mm.18522

3D structure databases

ProteinModelPortalⁱ	P97742
SMRⁱ	P97742
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	198863, 2 interactors
IntActⁱ	P97742, 1 interactor
STRINGⁱ	10090.ENSMUSP00000025835

PTM databases

iPTMnetⁱ	P97742
PhosphoSitePlusⁱ	P97742
SwissPalmⁱ	P97742

Proteomic databases

EPDⁱ	P97742
PaxDbⁱ	P97742
PeptideAtlasⁱ	P97742
PRIDEⁱ	P97742

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	12894
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENSMUST00000025835; ENSMUSP00000025835; ENSMUSG00000024900
GeneIDⁱ	12894
KEGGⁱ	mmu:12894
UCSCⁱ	uc008fwf.2 mouse

Organism-specific databases

CTDⁱ	1374
MGIⁱ	MGI:1098296 Cpt1a

Phylogenomic databases

eggNOGⁱ	KOG3717 Eukaryota ENOG410XNZ9 LUCA
GeneTreeⁱ	ENSGT00760000119220
HOGENOMⁱ	HOG000233542
HOVERGENⁱ	HBG003458
InParanoidⁱ	P97742
KOⁱ	K08765
OMAⁱ	ASHMWEN
OrthoDBⁱ	EOG091G026C
PhylomeDBⁱ	P97742
TreeFamⁱ	TF313836

Enzyme and pathway databases

UniPathwayⁱ	UPA00659
Reactomeⁱ	R-MMU-200425 Import of palmitoyl-CoA into the mitochondrial matrix R-MMU-5362517 Signaling by Retinoic Acid

Miscellaneous databases

Protein Ontology More...PROⁱ	PR:P97742
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSMUSG00000024900 Expressed in 329 organ(s), highest expression level in adrenal gland
CleanExⁱ	MM_CPT1A
ExpressionAtlasⁱ	P97742 baseline and differential
Genevisibleⁱ	P97742 MM

Family and domain databases

InterProⁱ	View protein in InterPro IPR000542 Carn_acyl_trans IPR039551 Cho/carn_acyl_trans IPR032476 CPT_N
PANTHERⁱ	PTHR22589 PTHR22589, 1 hit
Pfamⁱ	View protein in Pfam PF00755 Carn_acyltransf, 1 hit PF16484 CPT_N, 1 hit
PROSITEⁱ	View protein in PROSITE PS00439 ACYLTRANSF_C_1, 1 hit PS00440 ACYLTRANSF_C_2, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	CPT1A_MOUSE
Accessionⁱ	P97742Primary (citable) accession number: P97742 Secondary accession number(s): O35288 , Q80SW3, Q8BP98, Q8C7H8
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	November 1, 1997
	Last sequence update:	July 5, 2004
	Last modified:	November 7, 2018
	This is version 159 of the entry and version 4 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program

Miscellaneousⁱ

Keywords - Technical termⁱ

Complete proteome, Reference proteome

Documents

SIMILARITY comments
Index of protein domains and families
MGD cross-references
Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
PATHWAY comments
Index of metabolic and biosynthesis pathways

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UniParc

Help

UniRef

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Annotation systems

Supporting data

UniProtKB - P97742 (CPT1A_MOUSE)

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Carnitine O-palmitoyltransferase 1, liver isoform

Select a section on the left to see content.

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: fatty acid beta-oxidation

Sites

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

Enzyme and pathway databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Mitochondrion

Mitochondrion

Other locations

Topology

Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Amino acid modifications

Keywords - PTMi

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

GO - Molecular functioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Region

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Keywords - Domaini

Phylogenomic databases

Family and domain databases

Experimental Info

Sequence databases

Genome annotation databases

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

3D structure databases

Protein-protein interaction databases

PTM databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Documents

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Functionⁱ

Pathwayⁱ: fatty acid beta-oxidation

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

GO - Molecular functionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Domainⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ