EC Number |
Protein Variants |
Reference |
---|
1.2.4.4 | H291A |
site-directed mutagenesis, mutation of a alpha-subunit residue, highly reduced activity compared to the wild-type E1b component |
656171 |
1.2.4.4 | H291N |
site-directed mutagenesis, mutation of a alpha-subunit residue, highly reduced activity compared to the wild-type E1b component |
656171 |
1.2.4.4 | H291Q |
site-directed mutagenesis, mutation of a alpha-subunit residue, highly reduced activity compared to the wild-type E1b component |
656171 |
1.2.4.4 | H292A |
inactive enzyme, no binding of 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 | 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 | more |
missense mutation in E1b subunits that cause maple syrup urine disease: R114W-alpha, T166M-alpha, R220W-alpha. N222S-alpha. M64T-alpha. G204S-alpha. A208T-alpha. T265R-alpha, I281T-alpha, N126Y-beta, Q145K-alpha, H156R-beta, A209D-alpha, A240P-alpha. G245R-alpha, R252H-alpha, F364C-alpha, Y368C-alpha and Y393N-alpha |
349040 |
1.2.4.4 | more |
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 |
687543 |