| Identification |
|---|
| BMDB Protein ID
| BMDBP00638 |
| Secondary Accession Numbers
| |
| 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
|
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
MAAVAAFAGWLLRLRAAGADGPWRRLCGAGLSRGFLQSASAYGAAAQRRQVAHFTFQPDP
EPVEYGQTQKMNLFQAVTSALDNSLAKDPTAVIFGEDVAFGGVFRCTVGLRDKYGKDRVF
NTPLCEQGIVGFGIGIAVTGATAIAEIQFADYIFPAFDQIVNEAAKYRYRSGDLFNCGSL
TIRSPWGCVGHGALYHSQSPEAFFAHCPGIKVVVPRSPFQAKGLLLSCIEDKNPCIFFEP
KILYRAAVEQVPVEPYNIPLSQAEVIQEGSDVTLVAWGTQVHVIREVAAMAQEKLGVSCE
VIDLRTILPWDVDTVCKSVIKTGRLLVSHEAPLTGGFASEISSTVQEECFLNLEAPISRV
CGYDTPFPHIFEPFYIPDKWKCYDALRKMINY
|
| External Links |
|---|
| 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 |
