Article
Destabilized Soluble SOD1 Species as Potential Determinants of Disease Severity in Familial Amyotrophic Lateral Sclerosis.
ACS chemical neuroscience - 15 Apr 2026
Hirata Yoko, Kobatake Yui, Koyama Hiroko, Furuta Kyoji, Takemori Hiroshi, Kamatari Yuji O
Abstract excerpt
Mutations in the Cu/Zn superoxide dismutase (SOD1) gene are linked to familial amyotrophic lateral sclerosis (ALS), yet the identity of the toxic molecular species remains unclear. We investigated the relationship between protein misfolding and pathogenicity by expressing GFP-tagged wild-type and mutant SOD1 (A4V, H46R, G93A) in mouse hippocampal HT22 cells. Western blotting under nonreducing conditions suggested...
Topics
- Animals
- Amyotrophic Lateral Sclerosis
- Superoxide Dismutase-1
- Mice
- Humans
- Superoxide Dismutase
- Protein Folding
- Mutation
- Cell Line
- Hippocampus
- Severity of Illness Index
