Article
SIRT1 overexpression ameliorates a mouse model of SOD1-linked amyotrophic lateral sclerosis via HSF1/HSP70i chaperone system.
Molecular brain - 29 Aug 2014
Watanabe Seiji, Ageta-Ishihara Natsumi, Nagatsu Shinji, Takao Keizo, Komine Okiru, Endo Fumito, Miyakawa Tsuyoshi, Misawa Hidemi, Takahashi Ryosuke, Kinoshita Makoto, Yamanaka Koji
Abstract excerpt
BACKGROUND: Dominant mutations in superoxide dismutase 1 (SOD1) cause degeneration of motor neurons in a subset of inherited amyotrophic lateral sclerosis (ALS). The pathogenetic process mediated by misfolded and/or aggregated mutant SOD1 polypeptides is hypothesized to be suppressed by protein refolding. This genetic study is aimed to test whether mutant SOD1-mediated ALS pathology recapitulated in mice could be...
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