Identification |
HMDB Protein ID
| CDBP02231 |
Secondary Accession Numbers
| Not Available |
Name
| Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial |
Description
| Not Available |
Synonyms
|
- BCKAD-E2
- BCKADE2
- Branched-chain alpha-keto acid dehydrogenase complex component E2
- Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex
- Dihydrolipoamide branched chain transacylase
- Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase
|
Gene Name
| DBT |
Protein Type
| Enzyme |
Biological Properties |
General Function
| Involved in acyltransferase activity |
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).
|
GO Classification
|
Biological Process |
fatty-acyl-CoA biosynthetic process |
branched-chain amino acid catabolic process |
cellular nitrogen compound metabolic process |
Cellular Component |
microtubule cytoskeleton |
mitochondrial alpha-ketoglutarate dehydrogenase complex |
mitochondrial nucleoid |
Function |
binding |
catalytic activity |
transferase activity |
transferase activity, transferring acyl groups |
transferase activity, transferring acyl groups other than amino-acyl groups |
acyltransferase activity |
protein binding |
cofactor binding |
dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity |
Molecular Function |
dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity |
cofactor binding |
Process |
metabolic process |
cellular metabolic process |
fatty-acyl-coa metabolic process |
fatty-acyl-coa biosynthetic process |
cofactor metabolic process |
coenzyme metabolic process |
acyl-coa metabolic process |
|
Cellular Location
|
- Mitochondrion matrix
|
Pathways
|
Name | SMPDB/Pathwhiz | KEGG | Threonine and 2-Oxobutanoate Degradation | | Not Available | Valine, Leucine and Isoleucine Degradation | | | Beta-Ketothiolase Deficiency | | Not Available | 2-Methyl-3-Hydroxybutryl CoA Dehydrogenase Deficiency | | Not Available | Propionic Acidemia | | Not Available |
|
Gene Properties |
Chromosome Location
| 1 |
Locus
| 1p31 |
SNPs
| DBT |
Gene Sequence
|
>1449 bp
ATGGCTGCAGTCCGTATGCTGAGAACCTGGAGCAGGAATGCGGGGAAGCTGATTTGTGTT
CGCTATTTTCAAACATGTGGTAATGTTCATGTTTTGAAGCCAAATTATGTGTGTTTCTTT
GGTTATCCTTCATTCAAGTATAGTCATCCACATCACTTCCTGAAAACAACTGCTGCTCTC
CGTGGACAGGTTGTTCAGTTCAAGCTCTCAGACATTGGAGAAGGGATTAGAGAAGTAACT
GTTAAAGAATGGTATGTAAAAGAAGGAGATACAGTGTCTCAGTTTGATAGCATCTGTGAA
GTTCAAAGTGATAAAGCTTCTGTTACCATCACTAGTCGTTATGATGGAGTCATTAAAAAA
CTCTATTATAATCTAGACGATATTGCCTATGTGGGGAAGCCATTAGTAGACATAGAAACG
GAAGCTTTAAAAGATTCAGAAGAAGATGTTGTTGAAACTCCTGCAGTGTCTCATGATGAA
CATACACACCAAGAGATAAAGGGCCGAAAAACACTGGCAACTCCTGCAGTTCGCCGTCTG
GCAATGGAAAACAATATTAAGCTGAGTGAAGTTGTTGGCTCAGGAAAAGATGGCAGAATA
CTTAAAGAAGATATCCTCAACTATTTGGAAAAGCAGACAGGAGCTATATTGCCTCCTTCA
CCCAAAGTTGAAATTATGCCACCTCCACCAAAGCCAAAAGACATGACTGTTCCTATACTA
GTATCAAAACCTCCGGTATTCACAGGCAAAGACAAAACAGAACCCATAAAAGGCTTTCAA
AAAGCAATGGTCAAGACTATGTCTGCAGCCCTGAAGATACCTCATTTTGGTTATTGTGAT
GAGATTGACCTTACTGAACTGGTTAAGCTCCGAGAAGAATTAAAACCCATTGCATTTGCT
CGTGGAATTAAACTCTCCTTTATGCCTTTCTTCTTAAAGGCTGCTTCCTTGGGATTACTA
CAGTTTCCTATCCTTAACGCTTCTGTGGATGAAAACTGCCAGAATATAACATATAAGGCT
TCTCATAACATTGGGATAGCAATGGATACTGAGCAGGGTTTGATTGTCCCTAATGTGAAA
AATGTTCAGATCTGCTCTATATTTGACATCGCCACTGAACTGAACCGCCTCCAGAAATTG
GGCTCTGTGGGTCAGCTCAGCACCACTGATCTTACAGGAGGAACATTTACTCTTTCCAAC
ATTGGATCAATTGGTGGTACCTTTGCCAAACCAGTGATAATGCCACCTGAAGTAGCCATT
GGGGCCCTTGGATCAATTAAGGCCATTCCCCGATTTAACCAGAAAGGAGAAGTATATAAG
GCACAGATAATGAATGTGAGCTGGTCAGCTGATCACAGAGTTATTGATGGTGCTACAATG
TCACGCTTCTCCAATTTGTGGAAATCCTATTTAGAAAACCCAGCTTTTATGCTACTAGAT
CTGAAATGA
|
Protein Properties |
Number of Residues
| 482 |
Molecular Weight
| 53486.635 |
Theoretical pI
| 8.518 |
Pfam Domain Function
|
|
Signals
|
Not Available
|
Transmembrane Regions
|
Not Available
|
Protein Sequence
|
>Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
MAAVRMLRTWSRNAGKLICVRYFQTCGNVHVLKPNYVCFFGYPSFKYSHPHHFLKTTAAL
RGQVVQFKLSDIGEGIREVTVKEWYVKEGDTVSQFDSICEVQSDKASVTITSRYDGVIKK
LYYNLDDIAYVGKPLVDIETEALKDSEEDVVETPAVSHDEHTHQEIKGRKTLATPAVRRL
AMENNIKLSEVVGSGKDGRILKEDILNYLEKQTGAILPPSPKVEIMPPPPKPKDMTVPIL
VSKPPVFTGKDKTEPIKGFQKAMVKTMSAALKIPHFGYCDEIDLTELVKLREELKPIAFA
RGIKLSFMPFFLKAASLGLLQFPILNASVDENCQNITYKASHNIGIAMDTEQGLIVPNVK
NVQICSIFDIATELNRLQKLGSVGQLSTTDLTGGTFTLSNIGSIGGTFAKPVIMPPEVAI
GALGSIKAIPRFNQKGEVYKAQIMNVSWSADHRVIDGATMSRFSNLWKSYLENPAFMLLD
LK
|
External Links |
GenBank ID Protein
| 189053756 |
UniProtKB/Swiss-Prot ID
| P11182 |
UniProtKB/Swiss-Prot Entry Name
| ODB2_HUMAN |
PDB IDs
|
|
GenBank Gene ID
| AK313191 |
GeneCard ID
| DBT |
GenAtlas ID
| DBT |
HGNC ID
| HGNC:2698 |
References |
General References
| Not Available |