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Protein

Peroxisomal carnitine O-octanoyltransferase

Gene

CROT

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Beta-oxidation of fatty acids. The highest activity concerns the C6 to C10 chain length substrate. Converts the end product of pristanic acid beta oxidation, 4,8-dimethylnonanoyl-CoA, to its corresponding carnitine ester.1 Publication

Catalytic activityi

Octanoyl-CoA + L-carnitine = CoA + L-octanoylcarnitine.

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 sitei327Proton acceptorBy similarity1
Binding sitei406Coenzyme ABy similarity1
Binding sitei439CarnitineBy similarity1
Binding sitei441Carnitine; via carbonyl oxygenBy similarity1
Binding sitei452CarnitineBy similarity1

GO - Molecular functioni

  • carnitine O-octanoyltransferase activity Source: UniProtKB
  • signaling receptor binding Source: UniProtKB

GO - Biological processi

  • carnitine metabolic process Source: UniProtKB
  • coenzyme A metabolic process Source: UniProtKB
  • fatty acid beta-oxidation Source: UniProtKB
  • fatty acid beta-oxidation using acyl-CoA oxidase Source: Reactome
  • fatty acid metabolic process Source: UniProtKB
  • fatty acid transport Source: Ensembl
  • generation of precursor metabolites and energy Source: UniProtKB
  • medium-chain fatty acid metabolic process Source: UniProtKB
  • protein targeting to peroxisome Source: Reactome

Keywordsi

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

Enzyme and pathway databases

BRENDAi2.3.1.137 2681
ReactomeiR-HSA-389887 Beta-oxidation of pristanoyl-CoA
R-HSA-9033241 Peroxisomal protein import
UniPathwayiUPA00659

Protein family/group databases

TCDBi4.C.2.1.2 the carnitine o-acyl transferase (crat) family

Chemistry databases

SwissLipidsiSLP:000001055

Names & Taxonomyi

Protein namesi
Recommended name:
Peroxisomal carnitine O-octanoyltransferase (EC:2.3.1.137)
Short name:
COT
Gene namesi
Name:CROT
Synonyms:COT
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 7

Organism-specific databases

EuPathDBiHostDB:ENSG00000005469.11
HGNCiHGNC:2366 CROT
MIMi606090 gene
neXtProtiNX_Q9UKG9

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

Keywords - Cellular componenti

Peroxisome

Pathology & Biotechi

Organism-specific databases

DisGeNETi54677
OpenTargetsiENSG00000005469
PharmGKBiPA26887

Chemistry databases

ChEMBLiCHEMBL2206
DrugBankiDB02648 (3-Carboxy-2-(R)-Hydroxy-Propyl)-Trimethyl-Ammonium
DB00583 L-Carnitine

Polymorphism and mutation databases

BioMutaiCROT
DMDMi48429265

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
ChainiPRO_00002101691 – 612Peroxisomal carnitine O-octanoyltransferaseAdd BLAST612

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei1N-acetylmethionineCombined sources1
Modified residuei40N6-succinyllysineBy similarity1
Modified residuei57N6-succinyllysineBy similarity1
Modified residuei406N6-acetyllysine; alternateBy similarity1
Modified residuei406N6-succinyllysine; alternateBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ9UKG9
PaxDbiQ9UKG9
PeptideAtlasiQ9UKG9
PRIDEiQ9UKG9

PTM databases

iPTMnetiQ9UKG9
PhosphoSitePlusiQ9UKG9

Expressioni

Gene expression databases

BgeeiENSG00000005469
CleanExiHS_CROT
ExpressionAtlasiQ9UKG9 baseline and differential
GenevisibleiQ9UKG9 HS

Organism-specific databases

HPAiHPA019052
HPA019364
HPA019365

Interactioni

Subunit structurei

Monomer.1 Publication

GO - Molecular functioni

  • signaling receptor binding Source: UniProtKB

Protein-protein interaction databases

BioGridi120098, 11 interactors
IntActiQ9UKG9, 2 interactors
STRINGi9606.ENSP00000413575

Chemistry databases

BindingDBiQ9UKG9

Structurei

3D structure databases

ProteinModelPortaliQ9UKG9
SMRiQ9UKG9
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni410 – 417Coenzyme A bindingBy similarity8

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Motifi610 – 612Microbody targeting signalSequence analysis3

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi534 – 537Poly-Gly4

Sequence similaritiesi

Phylogenomic databases

eggNOGiKOG3718 Eukaryota
ENOG410ZE4S LUCA
GeneTreeiENSGT00760000119220
HOGENOMiHOG000122191
HOVERGENiHBG104403
InParanoidiQ9UKG9
KOiK05940
OMAiNEGRWKG
OrthoDBiEOG091G06ND
PhylomeDBiQ9UKG9
TreeFamiTF313836

Family and domain databases

InterProiView protein in InterPro
IPR000542 Carn_acyl_trans
PANTHERiPTHR22589 PTHR22589, 1 hit
PfamiView protein in Pfam
PF00755 Carn_acyltransf, 1 hit
PROSITEiView protein in PROSITE
PS00439 ACYLTRANSF_C_1, 1 hit
PS00440 ACYLTRANSF_C_2, 1 hit

Sequences (3)i

Sequence statusi: Complete.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q9UKG9-1) [UniParc]FASTAAdd 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
MENQLAKSTE ERTFQYQDSL PSLPVPSLEE SLKKYLESVK PFANQEEYKK
60 70 80 90 100
TEEIVQKFQS GIGEKLHQKL LERAKGKRNW LEEWWLNVAY LDVRIPSQLN
110 120 130 140 150
VNFAGPAAHF EHYWPPKEGT QLERGSITLW HNLNYWQLLR KEKVPVHKVG
160 170 180 190 200
NTPLDMNQFR MLFSTCKVPG ITRDSIMNYF RTESEGRSPN HIVVLCRGRA
210 220 230 240 250
FVFDVIHEGC LVTPPELLRQ LTYIHKKCHS EPDGPGIAAL TSEERTRWAK
260 270 280 290 300
AREYLIGLDP ENLALLEKIQ SSLLVYSMED SSPHVTPEDY SEIIAAILIG
310 320 330 340 350
DPTVRWGDKS YNLISFSNGV FGCNCDHAPF DAMIMVNISY YVDEKIFQNE
360 370 380 390 400
GRWKGSEKVR DIPLPEELIF IVDEKVLNDI NQAKAQYLRE ASDLQIAAYA
410 420 430 440 450
FTSFGKKLTK NKMLHPDTFI QLALQLAYYR LHGHPGCCYE TAMTRHFYHG
460 470 480 490 500
RTETMRSCTV EAVRWCQSMQ DPSVNLRERQ QKMLQAFAKH NKMMKDCSAG
510 520 530 540 550
KGFDRHLLGL LLIAKEEGLP VPELFTDPLF SKSGGGGNFV LSTSLVGYLR
560 570 580 590 600
VQGVVVPMVH NGYGFFYHIR DDRFVVACSA WKSCPETDAE KLVQLTFCAF
610
HDMIQLMNST HL
Length:612
Mass (Da):70,178
Last modified:June 7, 2004 - v2
Checksum:iBFC4E0A09B191038
GO
Isoform 2 (identifier: Q9UKG9-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     81-87: LEEWWLN → VFVVIIE
     88-612: Missing.

Show »
Length:87
Mass (Da):10,214
Checksum:i9806EC31C962CE35
GO
Isoform 3 (identifier: Q9UKG9-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     38-38: S → SVTRTCYQIRGLDPDAKRGFLDLTREGIQ

Note: No experimental confirmation available. Gene prediction based on cDNA data.
Show »
Length:640
Mass (Da):73,383
Checksum:i3215B8DD2DBB1F65
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti144V → L in AAF03234 (PubMed:10486279).Curated1
Sequence conflicti168V → G in AAD41654 (Ref. 2) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_04861294R → H. Corresponds to variant dbSNP:rs3827653Ensembl.1
Natural variantiVAR_048613474V → L. Corresponds to variant dbSNP:rs7785206Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_04695338S → SVTRTCYQIRGLDPDAKRGF LDLTREGIQ in isoform 3. Curated1
Alternative sequenceiVSP_04521381 – 87LEEWWLN → VFVVIIE in isoform 2. 1 Publication7
Alternative sequenceiVSP_04521488 – 612Missing in isoform 2. 1 PublicationAdd BLAST525

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF168793 mRNA Translation: AAF03234.1
AF073770 mRNA Translation: AAD41654.1
AC005045 Genomic DNA No translation available.
CH236949 Genomic DNA Translation: EAL24177.1
CH471091 Genomic DNA Translation: EAW76954.1
BC039004 mRNA Translation: AAH39004.1
BC051874 mRNA Translation: AAH51874.1
CCDSiCCDS47634.1 [Q9UKG9-3]
CCDS5604.1 [Q9UKG9-1]
CCDS59062.1 [Q9UKG9-2]
PIRiJC7101
RefSeqiNP_001137407.1, NM_001143935.1 [Q9UKG9-3]
NP_001230674.1, NM_001243745.1 [Q9UKG9-2]
NP_066974.2, NM_021151.3 [Q9UKG9-1]
UniGeneiHs.125039

Genome annotation databases

EnsembliENST00000331536; ENSP00000331981; ENSG00000005469 [Q9UKG9-1]
ENST00000412227; ENSP00000404867; ENSG00000005469 [Q9UKG9-2]
ENST00000419147; ENSP00000413575; ENSG00000005469 [Q9UKG9-3]
GeneIDi54677
KEGGihsa:54677
UCSCiuc003uis.4 human [Q9UKG9-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiOCTC_HUMAN
AccessioniPrimary (citable) accession number: Q9UKG9
Secondary accession number(s): A4D1D6
, E7EQF2, Q86V17, Q8IUW9, Q9Y6I2
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 11, 2001
Last sequence update: June 7, 2004
Last modified: May 23, 2018
This is version 150 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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

Complete proteome, Reference proteome

Documents

  1. Human chromosome 7
    Human chromosome 7: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. SIMILARITY comments
    Index of protein domains and families

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