Cellular analysis of SOD1 protein-aggregation propensity and toxicity: a case of ALS with slow progression harboring homozygous SOD1-D92G mutation

Cellular analysis of SOD1 protein-aggregation propensity and toxicity: a case of ALS with slow progression harboring homozygous SOD1-D92G mutation

Abstract Mutations within Superoxide dismutase 1 (SOD1) cause amyotrophic lateral sclerosis (ALS), accounting for approximately 20% of familial cases. The pathological feature is a loss of motor neurons with enhanced formation of intracellular misfolded SOD1. Homozygous SOD1-D90A in familial ALS has...

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Bibliographic Details
Main Authors: Masanori Sawamura, Keiko Imamura, Rie Hikawa, Takako Enami, Ayako Nagahashi, Hodaka Yamakado, Hidenori Ichijo, Takao Fujisawa, Hirofumi Yamashita, Sumio Minamiyama, Misako Kaido, Hiromi Wada, Makoto Urushitani, Haruhisa Inoue, Naohiro Egawa, Ryosuke Takahashi
Format: Article
Language:English
Published: Nature Portfolio 2022-07-01
Series:Scientific Reports
Online Access:https://doi.org/10.1038/s41598-022-16871-3

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https://doi.org/10.1038/s41598-022-16871-3

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