Article
Biochemical characterization of two mutants of human pyruvate dehydrogenase, F205L and T231A of the E1alpha subunit.
Journal of inherited metabolic disease - 1 Jan 2003
Wu Yong-Ge, Chen Wen-Yang, Zhang Zi-Wei, Yang Gui-Zheng, Li Wei, Duggleby Ronald G
Abstract excerpt
Mutations in the E1alpha subunit of the pyruvate dehydrogenase multienzyme complex may result in congenital lactic acidosis, but little is known about the consequences of these mutations at the enzymatic level. Here we characterize two mutants (F205L and T231A) of human pyruvate dehydrogenase in vitro, using the enzyme expressed in Escherichia coli. Wild-type and mutant proteins were purified successfully and...
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