Showing Protein 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial (BMDBP00638)

IdentificationBiological propertiesGene propertiesProtein propertiesExternal linksReferencesXMLShow 15 metabolites
Identification
BMDB Protein ID BMDBP00638
Secondary Accession Numbers
  • BMDBP03111
Name 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Synonyms Not Available
Gene Name BCKDHB
Protein Type Enzyme
Biological Properties
General Function Energy production and conversion
Specific Function The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Pathways Not Available
Reactions
3-Methyl-1-hydroxybutyl-ThPP + Lipoamide → S-(3-Methylbutanoyl)-dihydrolipoamide-E details
3-Methyl-1-hydroxybutyl-ThPP + 2-Methyl-1-hydroxypropyl-ThPP → Thiamine pyrophosphate details
2-Methyl-1-hydroxypropyl-ThPP → S-(2-Methylpropionyl)-dihydrolipoamide-E details
Lipoamide → S-(2-Methylpropionyl)-dihydrolipoamide-E details
Ketoleucine + Thiamine pyrophosphate → 3-Methyl-1-hydroxybutyl-ThPP + Carbon dioxide details
Alpha-ketoisovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide details
3-Methyl-2-oxovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxide details
2-Ketobutyric acid + Coenzyme A + NAD → Propionyl-CoA + NADH details
GO Classification Not Available
Cellular Location Not Available
Gene Properties
Chromosome Location 9
Locus Not Available
SNPs BCKDHB
Gene Sequence 
>1179 bp
ATGGCGGCTGTGGCGGCGTTCGCGGGCTGGCTGCTGCGGCTCCGTGCAGCCGGGGCCGAC
GGACCCTGGCGTCGGCTGTGTGGCGCGGGGCTGTCGAGGGGCTTCCTGCAGTCCGCCTCG
GCCTACGGGGCTGCGGCCCAGAGGCGGCAGGTGGCTCACTTCACTTTCCAGCCTGACCCG
GAGCCCGTGGAGTACGGGCAGACTCAGAAAATGAATCTCTTCCAGGCAGTAACAAGTGCC
TTAGATAACTCATTGGCCAAAGATCCTACGGCAGTAATATTTGGTGAAGACGTTGCCTTT
GGTGGAGTCTTTAGATGTACTGTCGGCTTGCGAGACAAGTATGGTAAAGATAGAGTTTTT
AATACCCCACTGTGTGAACAAGGAATCGTTGGATTTGGAATTGGAATCGCAGTCACCGGT
GCTACTGCCATAGCAGAAATTCAGTTTGCAGATTATATTTTCCCTGCTTTTGATCAGATT
GTTAATGAAGCTGCCAAGTATCGCTACCGGTCTGGGGACCTTTTTAATTGTGGAAGCCTC
ACCATCCGGTCCCCTTGGGGCTGTGTCGGCCACGGGGCTCTCTATCATTCCCAGAGTCCT
GAAGCTTTCTTTGCCCACTGCCCAGGAATCAAGGTGGTTGTACCCAGAAGCCCTTTCCAG
GCCAAGGGACTTCTTTTATCATGCATAGAGGATAAAAATCCTTGTATATTTTTTGAACCT
AAAATACTTTACAGGGCAGCAGTGGAGCAGGTTCCTGTAGAGCCATACAACATCCCCCTT
TCCCAAGCTGAAGTCATCCAAGAAGGGAGTGATGTCACTCTAGTTGCCTGGGGCACTCAG
GTTCATGTGATCCGAGAGGTGGATGCCATGGCTCAAGAGAAGCTTGGGGTGTCTTGTGAG
GTCATTGATCTGAGGACTATACTACCTTGGGATGTGGATACAGTTTGCAAGTCTGTGATC
AAAACAGGGCGACTGCTAGTAAGTCATGAGGCTCCTTTGACGGGCGGCTTTGCCTCTGAG
ATCAGCTCAACGGTTCAGGAAGAATGTTTCCTGAACCTGGAAGCTCCTATATCAAGGGTG
TGTGGGTACGATACACCGTTCCCTCACATTTTTGAACCGTTCTACATCCCAGACAAGTGG
AAGTGCTATGATGCCCTTCGAAAAATGATCAACTATTGA
Protein Properties
Number of Residues 392
Molecular Weight 42935.0
Theoretical pI 6.17
Pfam Domain Function Not Available
Signals
  • None
Transmembrane Regions Not Available
Protein Sequence 
>2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
MAAVAAFAGWLLRLRAAGADGPWRRLCGAGLSRGFLQSASAYGAAAQRRQVAHFTFQPDP
EPVEYGQTQKMNLFQAVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVF
NTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSL
TIRSPWGCVGHGALYHSQSPEAFFAHCPGIKVVVPRSPFQAKGLLLSCIEDKNPCIFFEP
KILYRAAVEQVPVEPYNIPLSQAEVIQEGSDVTLVAWGTQVHVIREVAAMAQEKLGVSCE
VIDLRTILPWDVDTVCKSVIKTGRLLVSHEAPLTGGFASEISSTVQEECFLNLEAPISRV
CGYDTPFPHIFEPFYIPDKWKCYDALRKMINY
GenBank ID Protein AAA30407.1
UniProtKB/Swiss-Prot ID P21839
UniProtKB/Swiss-Prot Entry Name ODBB_BOVIN
PDB IDs
GenBank Gene ID M33323
GeneCard ID BCKDHB
GenAtlas ID Not Available
HGNC ID Not Available
References
General References Not Available