Showing Protein 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (BMDBP00637)

IdentificationBiological propertiesGene propertiesProtein propertiesExternal linksReferencesXMLShow 16 metabolites
Identification
BMDB Protein ID BMDBP00637
Secondary Accession Numbers None
Name 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Synonyms Not Available
Gene Name BCKDHA
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
  • Propanoate Metabolism
  • Threonine and 2-Oxobutanoate Degradation
  • Valine, Leucine, and Isoleucine Degradation
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 18
Locus Not Available
SNPs BCKDHA
Gene Sequence 
>1368 bp
TTTTTTGGGTTGGTTAGATTTCATGCAGGGGTCAGCCAAGATGGCGATGGCGGTGGCGGT
TGCGGTGGCCAGGGTTTGGAGACCAAGTCGAGGCTTGGGACGGACGGGCCTCCCGCTCCT
GCGGCTGCTTGGGGCTCGTGGGCTGGCTAGATTTCACCCCCACAGGTGGCAGCAACAGCA
GCACTTCTCGTCCCTGGATGACAAGCCGCAGTTCCCAGGGGCCTCAGCGGAGTTCATAGA
CAAGCTCGAATTCATCCAGCCCAATGTCATCTCTGGGATCCCCATCTACCGGGTCATGGA
CCGGCAGGGCCAGATCATCAACCCCAGCGAGGATCCCCACCTGCCCCAGGAGAAGGTGCT
CAAATTCTACAAGAGCATGACCCTGCTCAACACCATGGACCGCATCCTCTATGAATCCCA
GAGGCAGGGCCGCATATCCTTCTACATGACCAACTATGGCGAAGAGGGGACACACGTGGG
GAGCGCAGCAGCCCTGGACGACACAGACCTGGTGTTTGGCCAGTACCGGGAGGCAGGTGT
GCTCATGTACCGGGACTACCCGTTGGAGCTGTTCATGGCCCAGTGCTACGGCAACGTGAG
CGACCTGGGCAAGGGGCGCCAGATGCCCGTCCACTACGGCTGCAGGGAGCGTCACTTCGT
CACCATCTCCTCTCCACTGGCCACGCAGATCCCCCAGGCGGTCGGGGCAGCCTACGCGGC
CAAGAGGGCCAACGCTAACAGGGTGGTCATCTGTTACTTTGGAGAGGGGGCGGCCAGTGA
GGGGGACGCCCACGCCGGCTTCAACTTCGCCGCCACCCTCGAGTGCCCCATCATCTTCTT
CTGTCGGAACAACGGCTACGCCATCTCCACGCCCACCTCGGAGCAGTACCGCGGGGACGG
CATAGCGGCTCGAGGCCCCGGGTACGGCATCCTGTCCATCCGCGTGGACGGCAATGATGT
GTTTGCCGTGTACAACGCCACCAAGGAGGCCCGGCGGCGGGCCGTGGCGGAGAACCAGCC
CTTCCTCATTGAGGCCATGACCTACAGGATCGGGCACCACAGCACCAGTGACGACAGCTC
GGCGTACCGCTCAGTGGACGAGGTCAACTACTGGGACAAGCAGGACCACCCCATCTCCCG
GCTGCGGCATCACCTGCAGAGCCGCGGCTGGTGGGACGACGAGCAGGAGAAGGCCTGGAG
GAAGCAGTCCCGCAAGAAGGTAATGGAGGCCTTTGAGCAGGCTGAGCGGAAGCTGAAGCC
CAACCCCAGCTTGATCTTCTCGGACGTGTATCAGGAGATGCCTGCCCAGCTCCGCAAGCA
GCAGGAGTCTCTGGCACGTCACCTCCAGACCTATGGTGAACACTACCC
Protein Properties
Number of Residues 455
Molecular Weight 51678.0
Theoretical pI 8.61
Pfam Domain Function Not Available
Signals
  • None
Transmembrane Regions Not Available
Protein Sequence 
>2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
MQGSAKMAMAVAVAVARVWRPSRGLGRTGLPLLRLLGARGLARFHPHRWQQQQHFSSLDD
KPQFPGASAEFIDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPQEKVLKFYKSMT
LLNTMDRILYESQRQGRISFYMTNYGEEGTHVGSAAALDDTDLVFGQYREAGVLMYRDYP
LELFMAQCYGNVSDLGKGRQMPVHYGCRERHFVTISSPLATQIPQAVGAAYAAKRANANR
VVICYFGEGAASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPTSEQYRGDGIAARGPG
YGILSIRVDGNDVFAVYNATKEARRRAVAENQPFLIEAMTYRIGHHSTSDDSSAYRSVDE
VNYWDKQDHPISRLRHHLQSRGWWDDEQEKAWRKQSRKKVMEAFEQAERKLKPNPSLIFS
DVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFEK
GenBank ID Protein AAA30595.1
UniProtKB/Swiss-Prot ID P11178
UniProtKB/Swiss-Prot Entry Name ODBA_BOVIN
PDB IDs
GenBank Gene ID J03759
GeneCard ID BCKDHA
GenAtlas ID Not Available
HGNC ID Not Available
References
General References Not Available