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MGP002179

Record overview

MGPD IDMGP002179
Gene ID5019
SpeciesHomo sapiens (Human)
Gene Name3-oxoacid CoA transferase 1
Gene Symbol OXCT1
SynonymsOXCT; SCOT;
Alternate namessuccinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial; 3-oxoacid CoA-transferase 1; 3-oxoacid-CoA transferase 1; SCOT-s; 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-oxoacid CoA transferase; succinyl-CoA:3-ketoacid-CoA transferase; succinyl-CoA:3-ketoacid-coenzyme A transferase 1, mitochondrial;
Chromosome5
Map Location5p13.1
EC Number2.8.3.5
SummaryThis 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]
OrthologsView 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:
MAALKLLSSGLRLCASARGSGATWYKGCVCSFSTSAHRHTKFYTDPVEAVKDIPDGATVLVGGFGLCGIPENLIDALLKTGVKGLTAVSNNAGVDNFGLG
LLLRSKQIKRMVSSYVGENAEFERQYLSGELEVELTPQGTLAERIRAGGAGVPAFYTPTGYGTLVQEGGSPIKYNKDGSVAIASKPREVREFNGQHFILE
EAITGDFALVKAWKADRAGNVIFRKSARNFNLPMCKAAETTVVEVEEIVDIGAFAPEDIHIPQIYVHRLIKGEKYEKRIERLSIRKEGDGEAKSAKPGDD
VRERIIKRAALEFEDGMYANLGIGIPLLASNFISPNITVHLQSENGVLGLGPYPRQHEADADLINAGKETVTILPGASFFSSDESFAMIRGGHVDLTMLG
AMQVSKYGDLANWMIPGKMVKGMGGAMDLVSSAKTKVVVTMEHSAKGNAHKIMEKCTLPLTGKQCVNRIITEKAVFDVDKKKGLTLIELWEGLTVDDVQK
STGCDFAVSPKLMPMQQIAN
 
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: 
TKFYTDPVEAVKDIPDGATVLVGGFGLCGIPENLIDALLKTGVKGLTAVSNNAGVDNFGLGLLLRSKQIKRMVSSYVGENAEFERQYLSGELEVELTPQG
TLAERIRAGGAGVPAFYTPTGYGTLVQEGGSPIKYNKDGSVAIASKPREVREFNGQHFILEEAITGDFALVKAWKADRAGNVIFRKSARNFNLPMCKAAE
TTVVEVEEIVDIGAFAPEDIHIPQIYVHRLIKGEKYEKRIERLSIRKEGDGEAKSAKPGDDVRERIIKRAALEFEDGMYANLGIGIPLLASNFISPNITV
HLQSENGVLGLGPYPRQHEADADLINAGKETVTILPGASFFSSDESFAMIRGGHVDLTMLGAMQVSKYGDLANWMIPGKMVKGMGGAMDLVSSAKTKVVV
TMEHSAKGNAHKIMEKCTLPLTGKQCVNRIITEKAVFDVDKKKGLTLIELWEGLTVDDVQKSTGCDFAVSPKLMPMQQIAN
 
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