Gene/Proteome Database (LMPD)

LMPD ID
LMP001815
Gene ID
5019
Species
Homo sapiens (Human)
Gene Name
3-oxoacid CoA transferase 1
Gene Symbol
OXCT1
Synonyms
OXCT; SCOT
Alternate Names
succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial; SCOT-s; 3-oxoacid CoA-transferase 1; 3-oxoacid-CoA transferase 1; succinyl CoA:3-oxoacid CoA transferase; succinyl-CoA:3-ketoacid-CoA transferase; somatic-type succinyl CoA:3-oxoacid CoA-transferase; somatic-type succinyl-CoA:3-oxoacid CoA-transferase; somatic-type succinyl-CoA:3-oxoacid-CoA-transferase; succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial
Chromosome
5
Map Location
5p13.1
EC Number
2.8.3.5
Summary
This gene encodes a member of the 3-oxoacid CoA-transferase gene family. The encoded protein is a homodimeric mitochondrial matrix enzyme that plays a central role in extrahepatic ketone body catabolism by catalyzing the reversible transfer of coenzyme A from succinyl-CoA to acetoacetate. Mutations in this gene are associated with succinyl CoA:3-oxoacid CoA transferase deficiency. [provided by RefSeq, Jul 2008]
Orthologs
View orthologs and multiple alignments for OXCT1

Proteins

succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial precursor
Refseq ID NP_000427
Protein GI 4557817
UniProt ID P55809
mRNA ID NM_000436
Length 520
RefSeq Status REVIEWED
MAALKLLSSGLRLCASARGSGATWYKGCVCSFSTSAHRHTKFYTDPVEAVKDIPDGATVLVGGFGLCGIPENLIDALLKTGVKGLTAVSNNAGVDNFGLGLLLRSKQIKRMVSSYVGENAEFERQYLSGELEVELTPQGTLAERIRAGGAGVPAFYTPTGYGTLVQEGGSPIKYNKDGSVAIASKPREVREFNGQHFILEEAITGDFALVKAWKADRAGNVIFRKSARNFNLPMCKAAETTVVEVEEIVDIGAFAPEDIHIPQIYVHRLIKGEKYEKRIERLSIRKEGDGEAKSAKPGDDVRERIIKRAALEFEDGMYANLGIGIPLLASNFISPNITVHLQSENGVLGLGPYPRQHEADADLINAGKETVTILPGASFFSSDESFAMIRGGHVDLTMLGAMQVSKYGDLANWMIPGKMVKGMGGAMDLVSSAKTKVVVTMEHSAKGNAHKIMEKCTLPLTGKQCVNRIITEKAVFDVDKKKGLTLIELWEGLTVDDVQKSTGCDFAVSPKLMPMQQIAN
transit_peptide: 1..39 calculated_mol_wt: 4086 peptide sequence: MAALKLLSSGLRLCASARGSGATWYKGCVCSFSTSAHRH mat_peptide: 40..520 product: succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial calculated_mol_wt: 52090 peptide sequence: TKFYTDPVEAVKDIPDGATVLVGGFGLCGIPENLIDALLKTGVKGLTAVSNNAGVDNFGLGLLLRSKQIKRMVSSYVGENAEFERQYLSGELEVELTPQGTLAERIRAGGAGVPAFYTPTGYGTLVQEGGSPIKYNKDGSVAIASKPREVREFNGQHFILEEAITGDFALVKAWKADRAGNVIFRKSARNFNLPMCKAAETTVVEVEEIVDIGAFAPEDIHIPQIYVHRLIKGEKYEKRIERLSIRKEGDGEAKSAKPGDDVRERIIKRAALEFEDGMYANLGIGIPLLASNFISPNITVHLQSENGVLGLGPYPRQHEADADLINAGKETVTILPGASFFSSDESFAMIRGGHVDLTMLGAMQVSKYGDLANWMIPGKMVKGMGGAMDLVSSAKTKVVVTMEHSAKGNAHKIMEKCTLPLTGKQCVNRIITEKAVFDVDKKKGLTLIELWEGLTVDDVQKSTGCDFAVSPKLMPMQQIAN

Gene Information

Entrez Gene ID
5019
Gene Name
3-oxoacid CoA transferase 1
Gene Symbol
OXCT1
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005759 TAS:Reactome C mitochondrial matrix
GO:0005739 IDA:UniProtKB C mitochondrion
GO:0008260 IMP:UniProtKB F 3-oxoacid CoA-transferase activity
GO:0042803 NAS:UniProtKB F protein homodimerization activity
GO:0060612 IEA:Ensembl P adipose tissue development
GO:0007420 IEA:Ensembl P brain development
GO:0046950 IMP:UniProtKB P cellular ketone body metabolic process
GO:0044255 TAS:Reactome P cellular lipid metabolic process
GO:0071229 IEA:Ensembl P cellular response to acid chemical
GO:0071333 IEA:Ensembl P cellular response to glucose stimulus
GO:0007507 IEA:Ensembl P heart development
GO:0046952 TAS:Reactome P ketone body catabolic process
GO:0042182 IEA:Ensembl P ketone catabolic process
GO:0032024 IEA:Ensembl P positive regulation of insulin secretion
GO:0014823 IEA:Ensembl P response to activity
GO:0042493 IEA:Ensembl P response to drug
GO:0045471 IEA:Ensembl P response to ethanol
GO:0009725 IEA:Ensembl P response to hormone
GO:0007584 IEA:Ensembl P response to nutrient
GO:0042594 IEA:Ensembl P response to starvation
GO:0044281 TAS:Reactome P small molecule metabolic process

BIOCYC Pathway Links

BIOCYC Pathway ID Description
PWY66-368 ketolysis

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_1861 Ketone body metabolism
REACT_59 Utilization of Ketone Bodies

Domain Information

InterPro Annotations

Accession Description
IPR012792 3-oxoacid CoA-transferase, subunit A
IPR012791 3-oxoacid CoA-transferase, subunit B
IPR014388 3-oxoacid_CoA-transferase
IPR004164 Coenzyme A transferase active site
IPR004163 Coenzyme A transferase binding site
IPR004165 Coenzyme A transferase family I

UniProt Annotations

Entry Information

Gene Name
3-oxoacid CoA transferase 1
Protein Entry
SCOT1_HUMAN
UniProt ID
P55809
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=P55809-1; Sequence=Displayed; Name=2; IsoId=P55809-2; Sequence=VSP_056310; Note=No experimental confirmation available.;
Catalytic Activity Succinyl-CoA + a 3-oxo acid = succinate + a 3- oxoacyl-CoA.
Disease Succinyl-CoA-3-ketoacid-CoA transferase deficiency (SCOTD) [MIM
Function Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate.
Pathway Ketone metabolism; succinyl-CoA degradation; acetoacetyl- CoA from succinyl-CoA
Similarity Belongs to the 3-oxoacid CoA-transferase family.
Subcellular Location Mitochondrion matrix.
Subunit Homodimer.
Tissue Specificity Abundant in heart, followed in order by kidney, brain, and muscle, whereas in liver it is undetectable; also detectable in leukocytes and fibroblasts.

Identical and Related Proteins

Unique RefSeq proteins for LMP001815 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
4557817 RefSeq NP_000427 520 succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial precursor

Identical Sequences to LMP001815 proteins

Reference Database Accession Length Protein Name
GI:4557817 GenBank AEU53498.1 520 Sequence 129 from patent US 8062891
GI:4557817 GenBank AFN90287.1 520 Sequence 220 from patent US 8198025
GI:4557817 GenBank AGO72451.1 520 Sequence 129 from patent US 8470972
GI:4557817 GenBank AGU32442.1 520 Sequence 129 from patent US 8507277
GI:4557817 GenBank AGX48346.1 520 Sequence 129 from patent US 8541550
GI:4557817 GenBank AIC49324.1 520 OXCT1, partial [synthetic construct]

Related Sequences to LMP001815 proteins

Reference Database Accession Length Protein Name
GI:4557817 DBBJ BAG35249.1 520 unnamed protein product [Homo sapiens]
GI:4557817 EMBL CAG33330.1 520 OXCT [Homo sapiens]
GI:4557817 GenBank AAP36984.1 521 Homo sapiens 3-oxoacid CoA transferase, partial [synthetic construct]
GI:4557817 GenBank AAX36917.1 521 3-oxoacid CoA transferase 1, partial [synthetic construct]
GI:4557817 GenBank AAX43515.1 521 3-oxoacid CoA transferase 1, partial [synthetic construct]
GI:4557817 RefSeq XP_003811027.1 520 PREDICTED: succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial [Pan paniscus]