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Protein

Carnitine O-palmitoyltransferase 1, liver isoform

Gene

Cpt1a

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

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 keyPosition(s)DescriptionActionsGraphical viewLength
Active sitei473Proton acceptorBy similarity1
Binding sitei589CarnitineBy similarity1
Binding sitei602CarnitineBy similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionAcyltransferase, Transferase
Biological processFatty acid metabolism, Lipid metabolism, Transport

Enzyme and pathway databases

ReactomeiR-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
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 19

Organism-specific databases

MGIiMGI:1098296 Cpt1a

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini2 – 47CytoplasmicSequence analysisAdd BLAST46
Transmembranei48 – 73HelicalSequence analysisAdd BLAST26
Topological domaini74 – 102Mitochondrial intermembraneSequence analysisAdd BLAST29
Transmembranei103 – 122HelicalSequence analysisAdd BLAST20
Topological domaini123 – 773CytoplasmicSequence analysisAdd BLAST651

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 keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemovedBy similarity
ChainiPRO_00002101602 – 773Carnitine O-palmitoyltransferase 1, liver isoformAdd BLAST772

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei2N-acetylalanineBy similarity1
Modified residuei282Nitrated tyrosineBy similarity1
Modified residuei588PhosphothreonineBy similarity1
Modified residuei589Nitrated tyrosineBy similarity1
Modified residuei604PhosphothreonineBy similarity1
Modified residuei741PhosphoserineBy similarity1
Modified residuei747PhosphoserineBy similarity1

Keywords - PTMi

Acetylation, Nitration, Phosphoprotein

Proteomic databases

EPDiP97742
PaxDbiP97742
PeptideAtlasiP97742
PRIDEiP97742

PTM databases

iPTMnetiP97742
PhosphoSitePlusiP97742
SwissPalmiP97742

Expressioni

Gene expression databases

BgeeiENSMUSG00000024900 Expressed in 329 organ(s), highest expression level in adrenal gland
CleanExiMM_CPT1A
ExpressionAtlasiP97742 baseline and differential
GenevisibleiP97742 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

Protein-protein interaction databases

BioGridi198863, 2 interactors
IntActiP97742, 1 interactor
STRINGi10090.ENSMUSP00000025835

Structurei

3D structure databases

ProteinModelPortaliP97742
SMRiP97742
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni555 – 567Coenzyme A bindingBy similarityAdd BLAST13

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

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG3717 Eukaryota
ENOG410XNZ9 LUCA
GeneTreeiENSGT00760000119220
HOGENOMiHOG000233542
HOVERGENiHBG003458
InParanoidiP97742
KOiK08765
OMAiASHMWEN
OrthoDBiEOG091G026C
PhylomeDBiP97742
TreeFamiTF313836

Family and domain databases

InterProiView protein in InterPro
IPR000542 Carn_acyl_trans
IPR039551 Cho/carn_acyl_trans
IPR032476 CPT_N
PANTHERiPTHR22589 PTHR22589, 1 hit
PfamiView protein in Pfam
PF00755 Carn_acyltransf, 1 hit
PF16484 CPT_N, 1 hit
PROSITEiView 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]FASTAAdd 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 keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti145R → C in AAH38395 (PubMed:15489334).Curated1
Sequence conflicti145R → C in AAH46383 (PubMed:15489334).Curated1
Sequence conflicti398Y → C in AAC31641 (PubMed:9680378).Curated1
Sequence conflicti531E → D in BAC36808 (PubMed:16141072).Curated1
Sequence conflicti630F → L in AAC31641 (PubMed:9680378).Curated1
Sequence conflicti732I → T in AAC31641 (PubMed:9680378).Curated1
Sequence conflicti760D → A in AAC31641 (PubMed:9680378).Curated1

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
CCDSiCCDS29395.1
RefSeqiNP_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
UniGeneiMm.18522

Genome annotation databases

EnsembliENSMUST00000025835; ENSMUSP00000025835; ENSMUSG00000024900
GeneIDi12894
KEGGimmu:12894
UCSCiuc008fwf.2 mouse

Similar proteinsi

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
CCDSiCCDS29395.1
RefSeqiNP_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
UniGeneiMm.18522

3D structure databases

ProteinModelPortaliP97742
SMRiP97742
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi198863, 2 interactors
IntActiP97742, 1 interactor
STRINGi10090.ENSMUSP00000025835

PTM databases

iPTMnetiP97742
PhosphoSitePlusiP97742
SwissPalmiP97742

Proteomic databases

EPDiP97742
PaxDbiP97742
PeptideAtlasiP97742
PRIDEiP97742

Protocols and materials databases

DNASUi12894
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000025835; ENSMUSP00000025835; ENSMUSG00000024900
GeneIDi12894
KEGGimmu:12894
UCSCiuc008fwf.2 mouse

Organism-specific databases

CTDi1374
MGIiMGI:1098296 Cpt1a

Phylogenomic databases

eggNOGiKOG3717 Eukaryota
ENOG410XNZ9 LUCA
GeneTreeiENSGT00760000119220
HOGENOMiHOG000233542
HOVERGENiHBG003458
InParanoidiP97742
KOiK08765
OMAiASHMWEN
OrthoDBiEOG091G026C
PhylomeDBiP97742
TreeFamiTF313836

Enzyme and pathway databases

UniPathwayi
UPA00659

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

Miscellaneous databases

PROiPR:P97742
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000024900 Expressed in 329 organ(s), highest expression level in adrenal gland
CleanExiMM_CPT1A
ExpressionAtlasiP97742 baseline and differential
GenevisibleiP97742 MM

Family and domain databases

InterProiView protein in InterPro
IPR000542 Carn_acyl_trans
IPR039551 Cho/carn_acyl_trans
IPR032476 CPT_N
PANTHERiPTHR22589 PTHR22589, 1 hit
PfamiView protein in Pfam
PF00755 Carn_acyltransf, 1 hit
PF16484 CPT_N, 1 hit
PROSITEiView protein in PROSITE
PS00439 ACYLTRANSF_C_1, 1 hit
PS00440 ACYLTRANSF_C_2, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiCPT1A_MOUSE
AccessioniPrimary (citable) accession number: P97742
Secondary accession number(s): O35288
, Q80SW3, Q8BP98, Q8C7H8
Entry historyiIntegrated 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 statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  3. PATHWAY comments
    Index of metabolic and biosynthesis pathways
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

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