Article
A cysteine residue affects the conformational state and neuronal toxicity of mutant SOD1 in mice: relevance to the pathogenesis of ALS.
Human molecular genetics - 15 Jun 2015
Nagano Seiichi, Takahashi Yoko, Yamamoto Kazuhiro, Masutani Hiroshi, Fujiwara Noriko, Urushitani Makoto, Araki Toshiyuki
Abstract excerpt
We previously showed by in vitro experiments that the cysteine residue (Cys111) near the dimer interface is critical for monomerization and resultant aggregate formation of mutant Cu, Zn-superoxide dismutase (SOD1) protein, which is toxic to motor neurons in familial amyotrophic lateral sclerosis...
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