EC Number | Cloned (Comment) | Organism |
---|---|---|
1.2.4.4 | expressed in Escherichia coli BL-21 cells | Homo sapiens |
EC Number | Crystallization (Comment) | Organism |
---|---|---|
1.2.4.4 | - |
Homo sapiens |
EC Number | Protein Variants | Comment | Organism |
---|---|---|---|
1.2.4.4 | D108N | less thermostable than wild type enzyme | Homo sapiens |
1.2.4.4 | D200A | less thermostable than wild type enzyme | Homo sapiens |
1.2.4.4 | D200A/D108N | the mutant is defective in folding and assembly and as no detectable overall activity | Homo sapiens |
1.2.4.4 | D200N | less thermostable than wild type enzyme | Homo sapiens |
1.2.4.4 | E198Q/D200N | the mutant is defective in folding and assembly and as no detectable overall activity | Homo sapiens |
1.2.4.4 | additional information | the hybrid E1b heterotetramer carrying one wild type, one S292Qalpha subunit, and two wild type beta subunits contains only one functional active site and exhibits nearly 40% of the wild type activity | Homo sapiens |
1.2.4.4 | S292Q | no activity | Homo sapiens |
EC Number | Inhibitors | Comment | Organism | Structure |
---|---|---|---|---|
1.2.4.4 | additional information | branched-chain alpha-keto acid dehydrogenase overall activity is nullified at 50% phosphorylation of E1b | Homo sapiens |
EC Number | KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|---|
1.2.4.4 | 0.119 | - |
2-oxo-isovalerate | wild type enzyme | Homo sapiens |
EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|---|
1.2.4.4 | mitochondrion | - |
Homo sapiens | 5739 | - |
EC Number | Organism | UniProt | Comment | Textmining |
---|---|---|---|---|
1.2.4.4 | Homo sapiens | - |
- |
- |
EC Number | Purification (Comment) | Organism |
---|---|---|
1.2.4.4 | His Trap HP column chromatography and Strep-Tactin Superflow gravity column chromatography | Homo sapiens |
EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|---|
1.2.4.4 | 2-oxoisovalerate + 2,6-dichlorophenolindophenol + CoA | - |
Homo sapiens | ? + CO2 + reduced 2,6-dichlorophenolindophenol | - |
? |
EC Number | Subunits | Comment | Organism |
---|---|---|---|
1.2.4.4 | heterotetramer | x-ray crystallography | Homo sapiens |
EC Number | Synonyms | Comment | Organism |
---|---|---|---|
1.2.4.4 | BCKDC | - |
Homo sapiens |
1.2.4.4 | branched-chain alpha-keto acid dehydrogenase | - |
Homo sapiens |
1.2.4.4 | E1b component | - |
Homo sapiens |
EC Number | Turnover Number Minimum [1/s] | Turnover Number Maximum [1/s] | Substrate | Comment | Organism | Structure |
---|---|---|---|---|---|---|
1.2.4.4 | 9.4 | - |
2-oxo-isovalerate | wild type enzyme | Homo sapiens |
EC Number | Cofactor | Comment | Organism | Structure |
---|---|---|---|---|
1.2.4.4 | thiamine diphosphate | dependent | Homo sapiens |