Article
A molecular basis for tetramer destabilization and aggregation of transthyretin Ala97Ser.
Protein science : a publication of the Protein Society - 1 Apr 2023
Wang Yi-Shiang, Huang Chun-Hsiang, Liou Gunn-Guang, Hsueh Hsueh-Wen, Liang Chi-Ting, Tseng Hsi-Ching, Huang Shing-Jong, Chao Chi-Chao, Hsieh Sung-Tsang, Tzeng Shiou-Ru
Abstract excerpt
Transthyretin (TTR)-related amyloidosis (ATTR) is a syndrome of diseases characterized by the extracellular deposition of fibrillar materials containing TTR variants. Ala97Ser (A97S) is the major mutation reported in Taiwanese ATTR patients. Here, we combine atomic resolution structural information together with the biochemical data to demonstrate that substitution of polar Ser for a small hydrophobic side chain...
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