id author title date pages extension mime words sentences flesch summary cache txt work_b7v6ptrc6zf73knxlijlxndi7q Jiu-Li Song Natural Osmolyte TrimethylamineN-Oxide Corrects Assembly Defects of Mutant Branched-chain α-Ketoacid Decarboxylase in Maple Syrup Urine Disease 2001 6 .pdf application/pdf 6245 556 68 This is the first report of the positive effect of TMAO in correcting protein assembly defects caused by human mutations. In contrast, activity of F364C-� mutant E1 was increased markedly from 1% of wild type without TMAO to 30% Refolding—The above results show that TMAO augments residual activity of folded MSUD mutant E1. address whether TMAO induced dissociation of mutant assembly intermediates, the Y368C-� E1, which comprised predominantly trapped heterodimers with a His6 tag on the E1� subunit, was immobilized on Ni2�-NTA resin and incubated with Urea-denatured wild type (WT) and mutant E1 were refolded in a buffer containing chaperonin GroEL/GroES and different concentrations of TMAO as Natural Osmolyte Trimethylamine N-Oxide Corrects Assembly Defects of Mutant Branched-chain α-Ketoacid Decarboxylase in Maple Syrup Urine Disease* Natural Osmolyte Trimethylamine N-Oxide Corrects Assembly Defects of Mutant Branched-chain α-Ketoacid Decarboxylase in Maple Syrup Urine Disease* ./cache/work_b7v6ptrc6zf73knxlijlxndi7q.pdf ./txt/work_b7v6ptrc6zf73knxlijlxndi7q.txt