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- W1960735897 abstract "Mutations in the copper/zinc superoxide dismutase (SOD1) gene produce an animal model of familial amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder. To test a new therapeutic strategy for ALS, we examined the effect of caspase inhibition in transgenic mice expressing mutant human SOD1 with a substitution of glycine to alanine in position 93 (mSOD1(G93A)). Intracerebroventricular administration of zVAD-fmk, a broad caspase inhibitor, delays disease onset and mortality. Moreover, zVAD-fmk inhibits caspase-1 activity as well as caspase-1 and caspase-3 mRNA up-regulation, providing evidence for a non-cell-autonomous pathway regulating caspase expression. Caspases play an instrumental role in neurodegeneration in transgenic mSOD1(G93A) mice, which suggests that caspase inhibition may have a protective role in ALS." @default.
- W1960735897 created "2016-06-24" @default.
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- W1960735897 date "2000-04-14" @default.
- W1960735897 modified "2023-10-10" @default.
- W1960735897 title "Functional Role of Caspase-1 and Caspase-3 in an ALS Transgenic Mouse Model" @default.
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- W1960735897 doi "https://doi.org/10.1126/science.288.5464.335" @default.
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