Article
Distinct amyloid fibril structures formed by ALS-causing SOD1 mutants G93A and D101N.
EMBO reports - 1 Oct 2025
Zhang Mu-Ya, Ma Yeyang, Wang Li-Qiang, Xia Wencheng, Li Xiang-Ning, Zhao Kun, Chen Jie, Li Dan, Zou Liangyu, Wang Zhengzhi, Liu Cong, Liang Yi
Abstract excerpt
Two hundred eight genetic mutations in SOD1 have been linked to amyotrophic lateral sclerosis (ALS). Of these, the G93A and D101N variants maintain much of their physiological function, closely resembling that of wild-type SOD1, and the SOD1-G93A transgenic mouse is the most extensively used mouse line in the study of ALS. In this study, we report two cryo-EM structures of amyloid fibrils formed by G93A and D101N...
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