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Identification
HMDB Protein ID HMDBP00714
Secondary Accession Numbers
  • 5988
Name Succinyl-CoA:3-ketoacid coenzyme A transferase 2, mitochondrial
Synonyms
  1. 3-oxoacid-CoA transferase 2A
  2. SCOT-t
  3. Testis-specific succinyl-CoA:3-oxoacid CoA-transferase
  4. 3-oxoacid CoA-transferase 2A
Gene Name OXCT2
Protein Type Enzyme
Biological Properties
General Function Involved in CoA-transferase activity
Specific 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 (By similarity).
Pathways
  • Butanoate metabolism
  • succinyl-CoA degradation
  • Synthesis and degradation of ketone bodies
  • Valine, leucine and isoleucine degradation
Reactions
Succinyl-CoA + a 3-oxo acid → Succinic acid + a 3-oxoacyl-CoA details
Succinyl-CoA + Acetoacetic acid → Succinic acid + Acetoacetyl-CoA details
GO Classification
Biological Process
ketone body catabolic process
Cellular Component
mitochondrion
microtubule-based flagellum
Component
mitochondrion
organelle
membrane-bounded organelle
intracellular membrane-bounded organelle
Function
catalytic activity
transferase activity
transferase activity, transferring sulfur-containing groups
coa-transferase activity
Molecular Function
3-oxoacid CoA-transferase activity
Process
metabolic process
catabolic process
cellular catabolic process
ketone body catabolic process
Cellular Location
  1. Mitochondrion
Gene Properties
Chromosome Location 1
Locus 1p34
SNPs OXCT2
Gene Sequence
>1554 bp
ATGGCGGCGCTGCGGCTCCTGGCGTCAGTGCTCGGGCGCGGGGTCCCCGCCGGCGGCTCA
GGGCTCGCGCTGTCCCAGGGCTGCGCCCGCTGCTTTGCCACCAGTCCCCGGCTCCGTGCC
AAGTTCTACGCGGACCCGGTGGAGATGGTGAAGGACATCTCTGACGGGGCGACCGTCATG
ATCGGGGGCTTCGGGCTCTGCGGGATCCCCGAGAACCTGATCGCCGCGCTGCTCAGGACC
CGCGTGAAAGACCTGCAGGTGGTCAGCAGCAACGTGGGCGTGGAGGACTTCGGCCTGGGC
CTCCTGCTGGCCGCCAGGCAGGTCCGTCGCATCGTCTGTTCCTACGTGGGCGAGAACACC
CTGTGCGAGAGCCAGTACCTGGCAGGAGAGCTGGAGCTGGAGCTCACGCCCCAGGGCACC
CTGGCCGAGCGCATCCGCGCGGGGGGCGCCGGGGTGCCCGCCTTCTACACCCCCACGGGC
TACGGGACCCTGGTCCAGGAAGGGGGCGCCCCCATCCGCTACACCCCGGACGGCCACCTG
GCGCTCATGAGCCAGCCCCGAGAGGTGAGGGAGTTCAACGGCGACCACTTCCTTTTGGAG
CGCGCCATCCGGGCAGACTTCGCCCTGGTGAAAGGGTGGAAGGCCGACCGGGCAGGAAAC
GTGGTCTTCAGGAGAAGCGCCCGCAATTTCAACGTGCCCATGTGCAAAGCTGCAGACGTC
ACGGCGGTGGAGGTGGAAGAGATCGTGGAGGTGGGGGCTTTCCCCCCAGAAGACATCCAC
GTTCCTAACATTTATGTAGATCGCGTGATAAAGGGGCAGAAATACGAGAAACGAATTGAG
CGCTTAACGATCCTGAAAGAGGAAGATGGAGACGCTGGAAAGGAAGAGGACGCCAGGACG
CGCATCATCAGACGCGCAGCTCTGGAATTTGAGGACGGCATGTACGCCAATCTGGGCATA
GGCATCCCCCTGCTGGCCAGCAACTTCATCAGTCCCAGCATGACTGTCCATCTTCACAGT
GAGAACGGGATCCTGGGCCTGGGCCCGTTTCCCACGGAAGATGAGGTGGATGCCGACCTC
ATCAATGCAGGCAAGCAGACGGTCACGGTGCTTCCCGGGGGCTGCTTCTTCGCCAGCGAC
GACTCCTTCGCCATGATCCGAGGGGGACACATCCAACTAACCATGCTTGGAGCCATGCAG
GTTTCCAAATACGGCGACCTGGCGAACTGGATGATCCCTGGCAAGAAGGTGAAAGGCATG
GGCGGTGCCATGGACTTGGTGTCCAGTCAGAAGACCAGAGTGGTGGTCACCATGCAGCAC
TGCACAAAGGACAACACCCCCAAGATCATGGAGAAATGCACCATGCCGCTGACCGGGAAG
CGGTGCGTGGACCGCATCATCACCGAGAAGGCCGTGTTTGACGTGCACAGGAAGAAAGAG
CTGACGCTGAGGGAGCTCTGGGAGGGCCTGACGGTGGACGACATCAAAAAGAGCACGGGG
TGTGCCTTTGCTGTGTCCCCGAACCTCAGGCCCATGCAGCAGGTGGCACCCTGA
Protein Properties
Number of Residues 517
Molecular Weight 56139.41
Theoretical pI 7.134
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Succinyl-CoA:3-ketoacid-coenzyme A transferase 2, mitochondrial
MAALRLLASVLGRGVPAGGSGLALSQGCARCFATSPRLRAKFYADPVEMVKDISDGATVM
IGGFGLCGIPENLIAALLRTRVKDLQVVSSNVGVEDFGLGLLLAARQVRRIVCSYVGENT
LCESQYLAGELELELTPQGTLAERIRAGGAGVPAFYTPTGYGTLVQEGGAPIRYTPDGHL
ALMSQPREVREFNGDHFLLERAIRADFALVKGWKADRAGNVVFRRSARNFNVPMCKAADV
TAVEVEEIVEVGAFPPEDIHVPNIYVDRVIKGQKYEKRIERLTILKEEDGDAGKEEDART
RIIRRAALEFEDGMYANLGIGIPLLASNFISPSMTVHLHSENGILGLGPFPTEDEVDADL
INAGKQTVTVLPGGCFFASDDSFAMIRGGHIQLTMLGAMQVSKYGDLANWMIPGKKVKGM
GGAMDLVSSQKTRVVVTMQHCTKDNTPKIMEKCTMPLTGKRCVDRIITEKAVFDVHRKKE
LTLRELWEGLTVDDIKKSTGCAFAVSPNLRPMQQVAP
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID Q9BYC2
UniProtKB/Swiss-Prot Entry Name SCOT2_HUMAN
PDB IDs Not Available
GenBank Gene ID AB050193
GeneCard ID OXCT2
GenAtlas ID OXCT2
HGNC ID HGNC:18606
References
General References
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  3. Tanaka H, Kohroki J, Iguchi N, Onishi M, Nishimune Y: Cloning and characterization of a human orthologue of testis-specific succinyl CoA: 3-oxo acid CoA transferase (Scot-t) cDNA. Mol Hum Reprod. 2002 Jan;8(1):16-23. [PubMed:11756565 ]