EC Number   |
Protein Variants |
Reference |
|---|
   1.2.4.4 | S293A |
10fold increased Km-value for 3-methyl-2-oxobutanoate, 2.8fold increased Km-value for 4-methyl-2-oxopentanoate, no change in Km-value for thiamine diphosphate |
349090 |
| 1.2.4.4 | more |
complete deletion of the bkdR open reading frame, produces fewer spores or the spores have reduced viability, is apparently not complemented in trans by the wild-type allele, is able to utilize leucine as sole carbon source and has no detectable growth defect, bkdR gene product acts as a repressor, rather than an activator of transcription |
674248 |
| 1.2.4.4 | more |
construction of transgenic Arabidopsis thaliana plants overexpressing the branched-chain alpha-keto acid dehydrogenase multienzyme complex BCKDC in chloroplasts via Agrobacterium tumefaciens transformation under control of the CaMV 35S promotor, accumulation of branched-chain acyl-CoAs and alteration of free amino acid composition in seeds, overview |
657104 |
| 1.2.4.4 | F364C |
E1alpha-missense mutation in type IA maple syrup urine disease that causes the occurence of exclusively alpha,beta dimers |
349091 |
| 1.2.4.4 | Y393N |
E1alpha-missense mutation in type IA maple syrup urine disease that causes the occurence of exclusively alpha,beta dimers |
349091 |
| 1.2.4.4 | more |
enzyme complex regulation by reversible phosphorylation is impaired in component E1b S292 and S302 mutants, no or residual acylation of the lipoic acid-bearing domain of component E2b, overview |
657370 |
| 1.2.4.4 | more |
hE1b-Tyr113Phe mutant, specifically affects the decarboxylation reaction, thiamine diphosphate appears hyperactive, is prone to paracatalytic inactivation |
677159 |
| 1.2.4.4 | D296A |
inactive enzyme, no ability of component E1 apoenzyme to reconstitute with thiamine diphosphate |
349090 |
| 1.2.4.4 | H292A |
inactive enzyme, no binding of thiamine diphosphate |
349090 |
| 1.2.4.4 | R288A |
inactive enzyme, no detectable phosphorylation |
349090 |
