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Article

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

Scientific reports - 25 Jul 2022

Sawamura Masanori, Imamura Keiko, Hikawa Rie, Enami Takako, Nagahashi Ayako, Yamakado Hodaka, Ichijo Hidenori, Fujisawa Takao, Yamashita Hirofumi, Minamiyama Sumio, Kaido Misako, Wada Hiromi, Urushitani Makoto, Inoue Haruhisa, Egawa Naohiro, Takahashi Ryosuke

Abstract excerpt

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 been reported to show slow disease progression. Here, we reported a rare case of a slowly progressive ALS patient harboring...

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Identifiers

PMID: 35879519
DOI: 10.1038/s41598-022-16871-3
PMCID: PMC9314329

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