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Article

Amyloid fibril structures and ferroptosis activation induced by ALS-causing SOD1 mutations.

Science advances - 1 Nov 2024

Wang Li-Qiang, Ma Yeyang, Zhang Mu-Ya, Yuan Han-Ye, Li Xiang-Ning, Xia Wencheng, Zhao Kun, Huang Xi, Chen Jie, Li Dan, Zou Liangyu, Wang Zhengzhi, Le Weidong, Liu Cong, Liang Yi

Abstract excerpt

Over 200 genetic mutations in copper-zinc superoxide dismutase (SOD1) have been linked to amyotrophic lateral sclerosis (ALS). Among these, two ALS-causing mutants, histidine-46→arginine (H46R) and glycine-85→arginine (G85R), exhibit a decreased capacity to bind metal ions. Here, we report two cryo-electron microscopy structures of amyloid fibrils formed by H46R and G85R. These mutations lead to the formation of...

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Methodology Data sources Editorial policy Privacy Delete account & data About Contact Helena Bioinformatics

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Identifiers

PMID: 39475611
DOI: 10.1126/sciadv.ado8499
PMCID: PMC11524188

Genes

SOD1

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