1.2.4.4 D108N less thermostable than wild type enzyme 687543 1.2.4.4 D200A less thermostable than wild type enzyme 687543 1.2.4.4 D200A/D108N the mutant is defective in folding and assembly and as no detectable overall activity 687543 1.2.4.4 D200N less thermostable than wild type enzyme 687543 1.2.4.4 D295A site-directed mutagenesis of an alpha-subunit residue of component E1b, increased activity compared to the wild-type 656230 1.2.4.4 D296A inactive enzyme, no ability of component E1 apoenzyme to reconstitute with thiamine diphosphate 349090 1.2.4.4 E198Q/D200N the mutant is defective in folding and assembly and as no detectable overall activity 687543 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 H146A site-directed mutagenesis, mutation of a beta'-subunit residue, no reductive acylation activity, low decarboxylation activity 656171 1.2.4.4 H147N site-directed mutagenesis, mutation of a beta'-subunit residue, no reductive acylation activity, low decarboxylation activity 656171