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.

Catalytic activityⁱ

Palmitoyl-CoA + L-carnitine = CoA + L-palmitoylcarnitine.2 Publications

Activity regulationⁱ

Inhibited by malonyl-CoA.1 Publication

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
Inferred from direct assayⁱ
- 11350182
identical protein binding Source: Ensembl
palmitoleoyltransferase activity Source: Ensembl

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

carnitine metabolic process
Source: UniProtKB
Inferred from direct assayⁱ
- 11350182
carnitine shuttle Source: Reactome
cellular response to fatty acid Source: Ensembl
circadian rhythm Source: Reactome
eating behavior Source: Ensembl
epithelial cell differentiation
Source: UniProtKB
Inferred from expression patternⁱ
- 21492153
fatty acid beta-oxidation
Source: ProtInc
Traceable author statementⁱ
- 7892212
glucose metabolic process Source: Ensembl
long-chain fatty acid metabolic process
Source: UniProtKB
Inferred from direct assayⁱ
- 11350182
positive regulation of fatty acid beta-oxidation Source: Ensembl
protein homooligomerization Source: Ensembl
regulation of insulin secretion Source: Ensembl
regulation of lipid metabolic process Source: Reactome
response to drug Source: Ensembl
response to organic cyclic compound Source: Ensembl
triglyceride metabolic process Source: Ensembl

View the complete GO annotation on QuickGO ...

Keywordsⁱ

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

Enzyme and pathway databases

BioCycⁱ	MetaCyc:HS03286-MONOMER
BRENDAⁱ	2.3.1.21 2681
Reactomeⁱ	R-HSA-1368082 RORA activates gene expression R-HSA-1989781 PPARA activates gene expression R-HSA-200425 Import of palmitoyl-CoA into the mitochondrial matrix R-HSA-5362517 Signaling by Retinoic Acid
SABIO-RKⁱ	P50416
SIGNORⁱ	P50416
UniPathwayⁱ	UPA00659

Protein family/group databases

TCDBⁱ	4.C.2.1.3 the carnitine o-acyl transferase (crat) family

TCDBⁱ

4.C.2.1.3 the carnitine o-acyl transferase (crat) family

Chemistry databases

SwissLipidsⁱ	SLP:000001056

SwissLipidsⁱ

SLP:000001056

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:CPT1
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [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 › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 11

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000110090.12
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:2328 CPT1A
MIMⁱ	600528 gene
neXtProtⁱ	NX_P50416

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

Involvement in diseaseⁱ

Carnitine palmitoyltransferase 1A deficiency (CPT1AD)8 Publications

The disease is caused by mutations affecting the gene represented in this entry.

Disease descriptionRare autosomal recessive metabolic disorder of long-chain fatty acid oxidation characterized by severe episodes of hypoketotic hypoglycemia usually occurring after fasting or illness. Onset is in infancy or early childhood.

Keywords - Diseaseⁱ

Disease mutation

Organism-specific databases

DisGeNET More...DisGeNETⁱ	1374
GeneReviewsⁱ	CPT1A
MalaCards human disease database More...MalaCardsⁱ	CPT1A
MIMⁱ	255120 phenotype
Open Targets More...OpenTargetsⁱ	ENSG00000110090
Orphanetⁱ	156 Carnitine palmitoyl transferase 1A deficiency
PharmGKBⁱ	PA26847

Chemistry databases

ChEMBLⁱ	CHEMBL1293194
Drug and drug target database More...DrugBankⁱ	DB01016 Glyburide DB00583 L-Carnitine DB01074 Perhexiline

Polymorphism and mutation databases

BioMutaⁱ	CPT1A
DMDMⁱ	56405343

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Initiator methionineⁱ		RemovedBy similarity
ChainⁱPRO_0000210159	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	P50416
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	P50416
PaxDbⁱ	P50416
PeptideAtlas More...PeptideAtlasⁱ	P50416
PRIDEⁱ	P50416
ProteomicsDBⁱ	56220 56221 [P50416-2]

PTM databases

iPTMnetⁱ	P50416
PhosphoSitePlusⁱ	P50416
SwissPalmⁱ	P50416

Expressionⁱ

Tissue specificityⁱ

Strong expression in kidney and heart, and lower in liver and skeletal muscle.

Inductionⁱ

Up-regulated by fatty acids.1 Publication

Gene expression databases

Bgeeⁱ	ENSG00000110090 Expressed in 227 organ(s), highest expression level in colonic mucosa
CleanExⁱ	HS_CPT1A
ExpressionAtlasⁱ	P50416 baseline and differential
Genevisibleⁱ	P50416 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA008835

HPAⁱ

HPA008835

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: Ensembl

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

BioGridⁱ	107765, 56 interactors
IntActⁱ	P50416, 32 interactors
Molecular INTeraction database More...MINTⁱ	P50416
STRINGⁱ	9606.ENSP00000265641

Chemistry databases

BindingDBⁱ

P50416

Structureⁱ

Secondary structure

Legend: HelixTurnBeta strandPDB Structure known for this area

Feature key	Position(s)	DescriptionActions	Length
Helixⁱ	4 – 7	Combined sources	4
Beta strandⁱ	9 – 15	Combined sources	7
Beta strandⁱ	18 – 23	Combined sources	6
Helixⁱ	25 – 39	Combined sources	15

Show more details

3D structure databases

ProteinModelPortalⁱ	P50416
SMRⁱ	P50416
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.1 Publication

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	P50416
KEGG Orthology (KO) More...KOⁱ	K08765
OMAⁱ	ASHMWEN
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G026C
PhylomeDBⁱ	P50416
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

Sequences (2+)ⁱ

Sequence statusⁱ: Complete.

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

This entry describes 2 isoformsⁱ produced by alternative splicing. Align Add to basket

This entry has 2 described isoforms and 4 potential isoforms that are computationally mapped.Show all Align All

Isoform 1 (identifier: P50416-1) [UniParc]FASTA Add to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MAEAHQAVAF QFTVTPDGID LRLSHEALRQ IYLSGLHSWK KKFIRFKNGI 
        60         70         80         90        100
ITGVYPASPS SWLIVVVGVM TTMYAKIDPS LGIIAKINRT LETANCMSSQ 
       110        120        130        140        150
TKNVVSGVLF GTGLWVALIV TMRYSLKVLL SYHGWMFTEH GKMSRATKIW 
       160        170        180        190        200
MGMVKIFSGR KPMLYSFQTS LPRLPVPAVK DTVNRYLQSV RPLMKEEDFK 
       210        220        230        240        250
RMTALAQDFA VGLGPRLQWY LKLKSWWATN YVSDWWEEYI YLRGRGPLMV 
       260        270        280        290        300
NSNYYAMDLL YILPTHIQAA RAGNAIHAIL LYRRKLDREE IKPIRLLGST 
       310        320        330        340        350
IPLCSAQWER MFNTSRIPGE ETDTIQHMRD SKHIVVYHRG RYFKVWLYHD 
       360        370        380        390        400
GRLLKPREME QQMQRILDNT SEPQPGEARL AALTAGDRVP WARCRQAYFG 
       410        420        430        440        450
RGKNKQSLDA VEKAAFFVTL DETEEGYRSE DPDTSMDSYA KSLLHGRCYD 
       460        470        480        490        500
RWFDKSFTFV VFKNGKMGLN AEHSWADAPI VAHLWEYVMS IDSLQLGYAE 
       510        520        530        540        550
DGHCKGDINP NIPYPTRLQW DIPGECQEVI ETSLNTANLL ANDVDFHSFP 
       560        570        580        590        600
FVAFGKGIIK KCRTSPDAFV QLALQLAHYK DMGKFCLTYE ASMTRLFREG 
       610        620        630        640        650
RTETVRSCTT ESCDFVRAMV DPAQTVEQRL KLFKLASEKH QHMYRLAMTG 
       660        670        680        690        700
SGIDRHLFCL YVVSKYLAVE SPFLKEVLSE PWRLSTSQTP QQQVELFDLE 
       710        720        730        740        750
NNPEYVSSGG GFGPVADDGY GVSYILVGEN LINFHISSKF SCPETDSHRF 
       760        770 
GRHLKEAMTD IITLFGLSSN SKK

Length:773

Mass (Da):88,368

Last modified:December 7, 2004 - v2

Checksum:ⁱE5DC9141B6301947

GO

Isoform 2 (identifier: P50416-2) [UniParc]FASTA Add to basket

The sequence of this isoform differs from the canonical sequence as follows:
746-773: DSHRFGRHLKEAMTDIITLFGLSSNSKK → GIISQGPSSDT

« Hide

        10         20         30         40         50
MAEAHQAVAF QFTVTPDGID LRLSHEALRQ IYLSGLHSWK KKFIRFKNGI 
        60         70         80         90        100
ITGVYPASPS SWLIVVVGVM TTMYAKIDPS LGIIAKINRT LETANCMSSQ 
       110        120        130        140        150
TKNVVSGVLF GTGLWVALIV TMRYSLKVLL SYHGWMFTEH GKMSRATKIW 
       160        170        180        190        200
MGMVKIFSGR KPMLYSFQTS LPRLPVPAVK DTVNRYLQSV RPLMKEEDFK 
       210        220        230        240        250
RMTALAQDFA VGLGPRLQWY LKLKSWWATN YVSDWWEEYI YLRGRGPLMV 
       260        270        280        290        300
NSNYYAMDLL YILPTHIQAA RAGNAIHAIL LYRRKLDREE IKPIRLLGST 
       310        320        330        340        350
IPLCSAQWER MFNTSRIPGE ETDTIQHMRD SKHIVVYHRG RYFKVWLYHD 
       360        370        380        390        400
GRLLKPREME QQMQRILDNT SEPQPGEARL AALTAGDRVP WARCRQAYFG 
       410        420        430        440        450
RGKNKQSLDA VEKAAFFVTL DETEEGYRSE DPDTSMDSYA KSLLHGRCYD 
       460        470        480        490        500
RWFDKSFTFV VFKNGKMGLN AEHSWADAPI VAHLWEYVMS IDSLQLGYAE 
       510        520        530        540        550
DGHCKGDINP NIPYPTRLQW DIPGECQEVI ETSLNTANLL ANDVDFHSFP 
       560        570        580        590        600
FVAFGKGIIK KCRTSPDAFV QLALQLAHYK DMGKFCLTYE ASMTRLFREG 
       610        620        630        640        650
RTETVRSCTT ESCDFVRAMV DPAQTVEQRL KLFKLASEKH QHMYRLAMTG 
       660        670        680        690        700
SGIDRHLFCL YVVSKYLAVE SPFLKEVLSE PWRLSTSQTP QQQVELFDLE 
       710        720        730        740        750
NNPEYVSSGG GFGPVADDGY GVSYILVGEN LINFHISSKF SCPETGIISQ 

GPSSDT

Show »

Length:756

Mass (Da):86,239

Checksum:ⁱC7B0ED8A26285110

GO

Computationally mapped potential isoform sequencesⁱ

There are 4 potential isoforms mapped to this entry.BLAST Align Show all Add to basket

Entry	Entry name	Protein names	Gene names	Length	Annotation

H3BUV7	H3BUV7_HUMAN	Carnitine O-palmitoyltransferase 1,... Carnitine O-palmitoyltransferase 1, liver isoform	CPT1A	134	Annotation score:
H3BMD2	H3BMD2_HUMAN	Carnitine O-palmitoyltransferase 1,... Carnitine O-palmitoyltransferase 1, liver isoform	CPT1A	78	Annotation score:
H3BP22	H3BP22_HUMAN	Carnitine O-palmitoyltransferase 1,... Carnitine O-palmitoyltransferase 1, liver isoform	CPT1A	117	Annotation score:
H3BUJ0	H3BUJ0_HUMAN	Carnitine O-palmitoyltransferase 1,... Carnitine O-palmitoyltransferase 1, liver isoform	CPT1A	93	Annotation score:

Experimental Info

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Sequence conflictⁱ	479	P → Q in AAC41748 (PubMed:7892212).Curated		1
Sequence conflictⁱ	568	A → T in AAC41748 (PubMed:7892212).Curated		1

Natural variant

Feature key	Position(s)	DescriptionActions	Length
Natural variantⁱVAR_020546	123	R → C in CPT1AD. 1 PublicationCorresponds to variant dbSNP:rs80356775EnsemblClinVar.	1
Natural variantⁱVAR_020547	275	A → T3 PublicationsCorresponds to variant dbSNP:rs2229738EnsemblClinVar.	1
Natural variantⁱVAR_020548	304	C → W in CPT1AD. 1 PublicationCorresponds to variant dbSNP:rs80356789EnsemblClinVar.	1
Natural variantⁱVAR_020549	314	T → I in CPT1AD. 1 PublicationCorresponds to variant dbSNP:rs80356776EnsemblClinVar.	1
Natural variantⁱVAR_046767	316	R → G in CPT1AD. 1 PublicationCorresponds to variant dbSNP:rs80356796EnsemblClinVar.	1
Natural variantⁱVAR_046768	343	F → V in CPT1AD. 1 PublicationCorresponds to variant dbSNP:rs80356783EnsemblClinVar.	1
Natural variantⁱVAR_020550	357	R → W in CPT1AD; decreased stability. 1 PublicationCorresponds to variant dbSNP:rs80356777EnsemblClinVar.	1
Natural variantⁱVAR_020551	360	E → G in CPT1AD; reduced protein levels. 1 PublicationCorresponds to variant dbSNP:rs80356787EnsemblClinVar.	1
Natural variantⁱVAR_020552	395	Missing in CPT1AD; loss of activity. 1 Publication	1
Natural variantⁱVAR_020553	414	A → V in CPT1AD; decreased activity. 2 PublicationsCorresponds to variant dbSNP:rs80356790EnsemblClinVar.	1
Natural variantⁱVAR_020554	454	D → G in CPT1AD. 1 PublicationCorresponds to variant dbSNP:rs80356778EnsemblClinVar.	1
Natural variantⁱVAR_046769	465	G → W in CPT1AD. 1 PublicationCorresponds to variant dbSNP:rs80356784EnsemblClinVar.	1
Natural variantⁱVAR_020555	479	P → L in CPT1AD; decreased activity. 1 PublicationCorresponds to variant dbSNP:rs80356779EnsemblClinVar.	1
Natural variantⁱVAR_020556	484	L → P in CPT1AD. 1 PublicationCorresponds to variant dbSNP:rs80356793EnsemblClinVar.	1
Natural variantⁱVAR_020557	498	Y → C in CPT1AD; decreased activity. 2 PublicationsCorresponds to variant dbSNP:rs80356791EnsemblClinVar.	1
Natural variantⁱVAR_020558	709	G → E in CPT1AD; loss of activity. 1 PublicationCorresponds to variant dbSNP:rs28936374EnsemblClinVar.	1
Natural variantⁱVAR_020559	710	G → E in CPT1AD; loss of activity. 2 PublicationsCorresponds to variant dbSNP:rs80356780EnsemblClinVar.	1

Alternative sequence

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Alternative sequenceⁱVSP_012167	746 – 773	DSHRF…SNSKK → GIISQGPSSDT in isoform 2. 2 PublicationsAdd BLAST		28

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	L39211 mRNA Translation: AAC41748.1 AJ420747 Genomic DNA Translation: CAD12625.1 AJ420748 Genomic DNA Translation: CAD59673.1 BT009791 mRNA Translation: AAP88793.1 BC000185 mRNA Translation: AAH00185.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS31624.1 [P50416-2] CCDS8185.1 [P50416-1]
PIRⁱ	I59351
NCBI Reference Sequences More...RefSeqⁱ	NP_001027017.1, NM_001031847.2 [P50416-2] NP_001867.2, NM_001876.3 [P50416-1] XP_016872709.1, XM_017017220.1 [P50416-1]
UniGeneⁱ	Hs.503043

Genome annotation databases

Ensemblⁱ	ENST00000265641; ENSP00000265641; ENSG00000110090 [P50416-1] ENST00000376618; ENSP00000365803; ENSG00000110090 [P50416-2] ENST00000539743; ENSP00000446108; ENSG00000110090 [P50416-1] ENST00000540367; ENSP00000439084; ENSG00000110090 [P50416-2]
GeneIDⁱ	1374
KEGGⁱ	hsa:1374
UCSC genome browser More...UCSCⁱ	uc001oof.5 human [P50416-1]

Keywords - Coding sequence diversityⁱ

Alternative splicing, Polymorphism

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
P50416	Carnitine palmitoyltransferase 1A (Liver), isoform CRA_a		HUMAN		773	UniRef100_P50416
CPT1A isoform 1 (Fragment)		PANTR		322
Carnitine O-palmitoyltransferase 1, liver isoform (Fragment)		HUMAN		117
Carnitine O-palmitoyltransferase 1, liver isoform (Fragment)		HUMAN		93
CPT1A isoform 4 (Fragment)		PANTR		118
+4

Protein	Similar proteins		Species	Score	Length	Source
P50416	Carnitine palmitoyltransferase 1A (Liver), isoform CRA_a		HUMAN		773	UniRef90_P50416
CPT1A isoform 1 (Fragment)		PANTR		322
Uncharacterized protein		PAPAN		764
Uncharacterized protein		RHIBE		762
Uncharacterized protein		MANLE		735
+13

Protein	Similar proteins		Species	Score	Length	Source
P50416	Carnitine O-palmitoyltransferase 1, liver isoform		RAT		773	UniRef50_P32198
Carnitine O-palmitoyltransferase 1, liver isoform		MOUSE		773
Carnitine O-palmitoyltransferase 1, muscle isoform		BOVIN		771
Carnitine O-palmitoyltransferase 1, muscle isoform		PIG		772
Carnitine O-palmitoyltransferase 1, muscle isoform		HUMAN		772
+780

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	L39211 mRNA Translation: AAC41748.1 AJ420747 Genomic DNA Translation: CAD12625.1 AJ420748 Genomic DNA Translation: CAD59673.1 BT009791 mRNA Translation: AAP88793.1 BC000185 mRNA Translation: AAH00185.1
CCDSⁱ	CCDS31624.1 [P50416-2] CCDS8185.1 [P50416-1]
PIRⁱ	I59351
RefSeqⁱ	NP_001027017.1, NM_001031847.2 [P50416-2] NP_001867.2, NM_001876.3 [P50416-1] XP_016872709.1, XM_017017220.1 [P50416-1]
UniGeneⁱ	Hs.503043

3D structure databases

Select the link destinations: Protein Data Bank Europe More...PDBeⁱ Protein Data Bank RCSB More...RCSB PDBⁱ Protein Data Bank Japan More...PDBjⁱ Links Updated	PDB entry	Method	Resolution (Å)	Chain	Positions	PDBsum
	2LE3	NMR	-	A	1-42	[»]
ProteinModelPortalⁱ	P50416
SMRⁱ	P50416
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	107765, 56 interactors
IntActⁱ	P50416, 32 interactors
MINTⁱ	P50416
STRINGⁱ	9606.ENSP00000265641

Chemistry databases

BindingDBⁱ	P50416
ChEMBLⁱ	CHEMBL1293194
DrugBankⁱ	DB01016 Glyburide DB00583 L-Carnitine DB01074 Perhexiline
SwissLipidsⁱ	SLP:000001056

Protein family/group databases

TCDBⁱ	4.C.2.1.3 the carnitine o-acyl transferase (crat) family

TCDBⁱ

4.C.2.1.3 the carnitine o-acyl transferase (crat) family

PTM databases

iPTMnetⁱ	P50416
PhosphoSitePlusⁱ	P50416
SwissPalmⁱ	P50416

Polymorphism and mutation databases

BioMutaⁱ	CPT1A
DMDMⁱ	56405343

Proteomic databases

EPDⁱ	P50416
MaxQBⁱ	P50416
PaxDbⁱ	P50416
PeptideAtlasⁱ	P50416
PRIDEⁱ	P50416
ProteomicsDBⁱ	56220 56221 [P50416-2]

Protocols and materials databases

The DNASU plasmid repository More...DNASUⁱ	1374
Structural Biology Knowledgebase	Search...

Genome annotation databases

Ensemblⁱ	ENST00000265641; ENSP00000265641; ENSG00000110090 [P50416-1] ENST00000376618; ENSP00000365803; ENSG00000110090 [P50416-2] ENST00000539743; ENSP00000446108; ENSG00000110090 [P50416-1] ENST00000540367; ENSP00000439084; ENSG00000110090 [P50416-2]
GeneIDⁱ	1374
KEGGⁱ	hsa:1374
UCSCⁱ	uc001oof.5 human [P50416-1]

Organism-specific databases

CTDⁱ	1374
DisGeNETⁱ	1374
EuPathDBⁱ	HostDB:ENSG00000110090.12
GeneCardsⁱ	CPT1A
GeneReviewsⁱ	CPT1A
HGNCⁱ	HGNC:2328 CPT1A
HPAⁱ	HPA008835
MalaCardsⁱ	CPT1A
MIMⁱ	255120 phenotype 600528 gene
neXtProtⁱ	NX_P50416
OpenTargetsⁱ	ENSG00000110090
Orphanetⁱ	156 Carnitine palmitoyl transferase 1A deficiency
PharmGKBⁱ	PA26847
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG3717 Eukaryota ENOG410XNZ9 LUCA
GeneTreeⁱ	ENSGT00760000119220
HOGENOMⁱ	HOG000233542
HOVERGENⁱ	HBG003458
InParanoidⁱ	P50416
KOⁱ	K08765
OMAⁱ	ASHMWEN
OrthoDBⁱ	EOG091G026C
PhylomeDBⁱ	P50416
TreeFamⁱ	TF313836

Enzyme and pathway databases

UniPathwayⁱ	UPA00659
BioCycⁱ	MetaCyc:HS03286-MONOMER
BRENDAⁱ	2.3.1.21 2681
Reactomeⁱ	R-HSA-1368082 RORA activates gene expression R-HSA-1989781 PPARA activates gene expression R-HSA-200425 Import of palmitoyl-CoA into the mitochondrial matrix R-HSA-5362517 Signaling by Retinoic Acid
SABIO-RKⁱ	P50416
SIGNORⁱ	P50416

Miscellaneous databases

ChiTaRSⁱ	CPT1A human
GenomeRNAiⁱ	1374
Protein Ontology More...PROⁱ	PR:P50416
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000110090 Expressed in 227 organ(s), highest expression level in colonic mucosa
CleanExⁱ	HS_CPT1A
ExpressionAtlasⁱ	P50416 baseline and differential
Genevisibleⁱ	P50416 HS

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_HUMAN
Accessionⁱ	P50416Primary (citable) accession number: P50416 Secondary accession number(s): Q8TCU0, Q9BWK0
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	October 1, 1996
	Last sequence update:	December 7, 2004
	Last modified:	November 7, 2018
	This is version 180 of the entry and version 2 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program
Disclaimer	Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousⁱ

Keywords - Technical termⁱ

3D-structure, Complete proteome, Reference proteome

Documents

SIMILARITY comments
Index of protein domains and families
Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations
Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
PATHWAY comments
Index of metabolic and biosynthesis pathways
PDB cross-references
Index of Protein Data Bank (PDB) cross-references
Human chromosome 11
Human chromosome 11: entries, gene names and cross-references to MIM

UniProtKB

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UniProtKB - P50416 (CPT1A_HUMAN)

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Carnitine O-palmitoyltransferase 1, liver isoform

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<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

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GO - Biological processi

Enzyme and pathway databases

Protein family/group databases

Chemistry 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

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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

Keywords - Diseasei

Organism-specific databases

Chemistry databases

Polymorphism and mutation databases

<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

Organism-specific 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

Chemistry 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

Secondary structure

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

Natural variant

Alternative sequence

Sequence databases

Genome annotation databases

Keywords - Coding sequence diversityi

<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

Chemistry databases

Protein family/group databases

PTM databases

Polymorphism and mutation databases

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Protocols and materials databases

Genome annotation databases

Organism-specific databases

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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

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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

Keywords - Diseaseⁱ

PTM / Processingⁱ

Keywords - PTMⁱ

Expressionⁱ

Interactionⁱ

GO - Molecular functionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Keywords - Domainⁱ

Keywords - Coding sequence diversityⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ