Article
Decreased thermodynamic stability as a crucial factor for familial amyloidotic polyneuropathy.
Journal of molecular biology - 5 Jul 2002
Niraula Tara Nath, Haraoka Katsuki, Ando Yukio, Li Hua, Yamada Hiroaki, Akasaka Kazuyuki
Abstract excerpt
A single mutation in the wild-type transthyretin (WT TTR) such as V30M causes a familial amyloidotic polyneuropathy disease. Comparison of the three-dimensional crystal structures of WT and V30M does not tell much about the reason. High-pressure NMR revealed that at neutral pH both WT and V30M exist as equilibrium between the native tetramer and the dissociated/unfolded monomer. The native tetramer is highly...
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