| 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
|
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
MQGSAKMAMAVAVAVARVWRPSRGLGRTGLPLLRLLGARGLARFHPHRWQQQQHFSSLDD
KPQFPGASAEFIDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPQEKVLKFYKSMT
LLNTMDRILYESQRQGRISFYMTNYGEEGTHVGSAAALDDTDLVFGQYREAGVLMYRDYP
LELFMAQCYGNVSDLGKGRQMPVHYGCRERHFVTISSPLATQIPQAVGAAYAAKRANANR
VVICYFGEGAASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPTSEQYRGDGIAARGPG
YGILSIRVDGNDVFAVYNATKEARRRAVAENQPFLIEAMTYRIGHHSTSDDSSAYRSVDE
VNYWDKQDHPISRLRHHLQSRGWWDDEQEKAWRKQSRKKVMEAFEQAERKLKPNPSLIFS
DVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFEK
|
| External Links |
|---|
| 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 |
