Gene/Proteome Database (LMPD)
LMPD ID
LMP000800
Gene ID
Species
Homo sapiens (Human)
Gene Name
carnitine O-octanoyltransferase
Gene Symbol
Synonyms
COT
Alternate Names
peroxisomal carnitine O-octanoyltransferase; peroxisomal carnitine acyltransferase
Chromosome
7
Map Location
7q21.1
EC Number
2.3.1.137
Summary
This gene encodes a member of the carnitine/choline acetyltransferase family. The encoded protein converts 4,8-dimethylnonanoyl-CoA to its corresponding carnitine ester. This transesterification occurs in the peroxisome and is necessary for transport of medium- and long- chain acyl-CoA molecules out of the peroxisome to the cytosol and mitochondria. The protein thus plays a role in lipid metabolism and fatty acid beta-oxidation. Alternatively spliced transcript variants have been described.[provided by RefSeq, Jan 2009]
Orthologs
Proteins
| peroxisomal carnitine O-octanoyltransferase isoform 1 | |
|---|---|
| Refseq ID | NP_001137407 |
| Protein GI | 221316602 |
| UniProt ID | Q9UKG9 |
| mRNA ID | NM_001143935 |
| Length | 640 |
| RefSeq Status | REVIEWED |
| MENQLAKSTEERTFQYQDSLPSLPVPSLEESLKKYLESVTRTCYQIRGLDPDAKRGFLDLTREGIQVKPFANQEEYKKTEEIVQKFQSGIGEKLHQKLLERAKGKRNWLEEWWLNVAYLDVRIPSQLNVNFAGPAAHFEHYWPPKEGTQLERGSITLWHNLNYWQLLRKEKVPVHKVGNTPLDMNQFRMLFSTCKVPGITRDSIMNYFRTESEGRSPNHIVVLCRGRAFVFDVIHEGCLVTPPELLRQLTYIHKKCHSEPDGPGIAALTSEERTRWAKAREYLIGLDPENLALLEKIQSSLLVYSMEDSSPHVTPEDYSEIIAAILIGDPTVRWGDKSYNLISFSNGVFGCNCDHAPFDAMIMVNISYYVDEKIFQNEGRWKGSEKVRDIPLPEELIFIVDEKVLNDINQAKAQYLREASDLQIAAYAFTSFGKKLTKNKMLHPDTFIQLALQLAYYRLHGHPGCCYETAMTRHFYHGRTETMRSCTVEAVRWCQSMQDPSVNLRERQQKMLQAFAKHNKMMKDCSAGKGFDRHLLGLLLIAKEEGLPVPELFTDPLFSKSGGGGNFVLSTSLVGYLRVQGVVVPMVHNGYGFFYHIRDDRFVVACSAWKSCPETDAEKLVQLTFCAFHDMIQLMNSTHL | |
| peroxisomal carnitine O-octanoyltransferase isoform 2 | |
|---|---|
| Refseq ID | NP_066974 |
| Protein GI | 31542325 |
| UniProt ID | Q9UKG9 |
| mRNA ID | NM_021151 |
| Length | 612 |
| RefSeq Status | REVIEWED |
| MENQLAKSTEERTFQYQDSLPSLPVPSLEESLKKYLESVKPFANQEEYKKTEEIVQKFQSGIGEKLHQKLLERAKGKRNWLEEWWLNVAYLDVRIPSQLNVNFAGPAAHFEHYWPPKEGTQLERGSITLWHNLNYWQLLRKEKVPVHKVGNTPLDMNQFRMLFSTCKVPGITRDSIMNYFRTESEGRSPNHIVVLCRGRAFVFDVIHEGCLVTPPELLRQLTYIHKKCHSEPDGPGIAALTSEERTRWAKAREYLIGLDPENLALLEKIQSSLLVYSMEDSSPHVTPEDYSEIIAAILIGDPTVRWGDKSYNLISFSNGVFGCNCDHAPFDAMIMVNISYYVDEKIFQNEGRWKGSEKVRDIPLPEELIFIVDEKVLNDINQAKAQYLREASDLQIAAYAFTSFGKKLTKNKMLHPDTFIQLALQLAYYRLHGHPGCCYETAMTRHFYHGRTETMRSCTVEAVRWCQSMQDPSVNLRERQQKMLQAFAKHNKMMKDCSAGKGFDRHLLGLLLIAKEEGLPVPELFTDPLFSKSGGGGNFVLSTSLVGYLRVQGVVVPMVHNGYGFFYHIRDDRFVVACSAWKSCPETDAEKLVQLTFCAFHDMIQLMNSTHL | |
| peroxisomal carnitine O-octanoyltransferase isoform 3 | |
|---|---|
| Refseq ID | NP_001230674 |
| Protein GI | 344217750 |
| UniProt ID | Q9UKG9 |
| mRNA ID | NM_001243745 |
| Length | 87 |
| RefSeq Status | REVIEWED |
| MENQLAKSTEERTFQYQDSLPSLPVPSLEESLKKYLESVKPFANQEEYKKTEEIVQKFQSGIGEKLHQKLLERAKGKRNWVFVVIIE | |
Gene Information
Entrez Gene ID
Gene Name
carnitine O-octanoyltransferase
Gene Symbol
Species
Homo sapiens
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0043231 | IDA:HPA | C | intracellular membrane-bounded organelle |
| GO:0005739 | IEA:Ensembl | C | mitochondrion |
| GO:0005782 | TAS:Reactome | C | peroxisomal matrix |
| GO:0005777 | IDA:UniProtKB | C | peroxisome |
| GO:0008458 | IDA:UniProtKB | F | carnitine O-octanoyltransferase activity |
| GO:0005102 | IPI:UniProtKB | F | receptor binding |
| GO:0009437 | ISS:UniProtKB | P | carnitine metabolic process |
| GO:0044255 | TAS:Reactome | P | cellular lipid metabolic process |
| GO:0015936 | ISS:UniProtKB | P | coenzyme A metabolic process |
| GO:0006635 | IDA:UniProtKB | P | fatty acid beta-oxidation |
| GO:0033540 | TAS:Reactome | P | fatty acid beta-oxidation using acyl-CoA oxidase |
| GO:0006631 | IMP:UniProtKB | P | fatty acid metabolic process |
| GO:0015908 | IEA:Ensembl | P | fatty acid transport |
| GO:0006091 | IDA:UniProtKB | P | generation of precursor metabolites and energy |
| GO:0051791 | IDA:UniProtKB | P | medium-chain fatty acid metabolic process |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
KEGG Pathway Links
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| REACT_17017 | Beta-oxidation of pristanoyl-CoA |
| REACT_16957 | Peroxisomal lipid metabolism |
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR000542 | Acyltransferase ChoActase/COT/CPT |
UniProt Annotations
Entry Information
Comments
| Comment Type | Description |
|---|---|
| Alternative Products | Event=Alternative splicing; Named isoforms=3; Name=1; IsoId=Q9UKG9-1; Sequence=Displayed; Name=2; IsoId=Q9UKG9-2; Sequence=VSP_045213, VSP_045214; Name=3; IsoId=Q9UKG9-3; Sequence=VSP_046953; Note=No experimental confirmation available. Gene prediction based on cDNA data.; |
| Catalytic Activity | Octanoyl-CoA + L-carnitine = CoA + L- octanoylcarnitine. |
| Function | 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. |
| Pathway | Lipid metabolism; fatty acid beta-oxidation. |
| Similarity | Belongs to the carnitine/choline acetyltransferase family. |
| Subcellular Location | Peroxisome . |
| Subunit | Monomer. |
Identical and Related Proteins
Unique RefSeq proteins for LMP000800 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 221316602 | RefSeq | NP_001137407 | 640 | peroxisomal carnitine O-octanoyltransferase isoform 1 |
| 31542325 | RefSeq | NP_066974 | 612 | peroxisomal carnitine O-octanoyltransferase isoform 2 |
| 344217750 | RefSeq | NP_001230674 | 87 | peroxisomal carnitine O-octanoyltransferase isoform 3 |
Identical Sequences to LMP000800 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:31542325 | GenBank | AAH39004.1 | 612 | Carnitine O-octanoyltransferase [Homo sapiens] |
| GI:344217750 | GenBank | AAH51874.1 | 87 | CROT protein [Homo sapiens] |
| GI:31542325 | GenBank | EAL24177.1 | 612 | carnitine O-octanoyltransferase [Homo sapiens] |
| GI:31542325 | GenBank | EAW76954.1 | 612 | carnitine O-octanoyltransferase [Homo sapiens] |
| GI:344217750 | GenBank | AIC58375.1 | 87 | CROT, partial [synthetic construct] |
| GI:31542325 | SwissProt | Q9UKG9.2 | 612 | RecName: Full=Peroxisomal carnitine O-octanoyltransferase; Short=COT [Homo sapiens] |
Related Sequences to LMP000800 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:344217750 | GenBank | AAD41654.1 | 612 | carnitine octanoyltransferase [Homo sapiens] |
| GI:31542325 | GenBank | AAD41654.1 | 612 | carnitine octanoyltransferase [Homo sapiens] |
| GI:31542325 | GenBank | AAF03234.1 | 612 | peroxisomal carnitine octanoyltransferase [Homo sapiens] |
| GI:344217750 | GenBank | AAF03234.1 | 612 | peroxisomal carnitine octanoyltransferase [Homo sapiens] |
| GI:344217750 | GenBank | AAH39004.1 | 612 | Carnitine O-octanoyltransferase [Homo sapiens] |
| GI:221316602 | GenBank | AAH39004.1 | 612 | Carnitine O-octanoyltransferase [Homo sapiens] |
| GI:344217750 | GenBank | EAL24177.1 | 612 | carnitine O-octanoyltransferase [Homo sapiens] |
| GI:344217750 | GenBank | EAW76954.1 | 612 | carnitine O-octanoyltransferase [Homo sapiens] |
| GI:31542325 | GenBank | ACM81822.1 | 656 | Sequence 7320 from patent US 6812339 |
| GI:31542325 | GenBank | JAA07694.1 | 612 | carnitine O-octanoyltransferase [Pan troglodytes] |
| GI:31542325 | GenBank | JAA21552.1 | 612 | carnitine O-octanoyltransferase [Pan troglodytes] |
| GI:31542325 | GenBank | JAA23183.1 | 612 | carnitine O-octanoyltransferase [Pan troglodytes] |
| GI:344217750 | RefSeq | NP_066974.2 | 612 | peroxisomal carnitine O-octanoyltransferase isoform 2 [Homo sapiens] |
| GI:221316602 | RefSeq | NP_066974.2 | 612 | peroxisomal carnitine O-octanoyltransferase isoform 2 [Homo sapiens] |
| GI:221316602 | RefSeq | XP_001106063.2 | 640 | PREDICTED: peroxisomal carnitine O-octanoyltransferase isoform 2 [Macaca mulatta] |
| GI:221316602 | RefSeq | XP_003252382.1 | 640 | PREDICTED: peroxisomal carnitine O-octanoyltransferase isoform 2 [Nomascus leucogenys] |
| GI:221316602 | RefSeq | XP_004045736.1 | 640 | PREDICTED: peroxisomal carnitine O-octanoyltransferase isoform 2 [Gorilla gorilla gorilla] |
| GI:221316602 | SwissProt | Q9UKG9.2 | 612 | RecName: Full=Peroxisomal carnitine O-octanoyltransferase; Short=COT [Homo sapiens] |