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- W1183617902 abstract "Neurofilaments represent a major cytoskeletal component in large neurons. There is growing evidence that perturbations in the normal metabolism of neurofilaments are associated with diseases. The abnormal accumulations of neurofilaments within neurons, often called spheroids or Lewy bodies, are frequently observed in human neurodegenerative disorders, including amyotrophic lateral sclerosis, Parkinson's disease, and Alzheimers disease. The first evidence that neurofilaments can play a causative role in neurodegenerative disease came from studies with transgenic mice that developed abnormal neurofilament accumulations as a result of overexpression of human NF-H or mouse NF-L proteins. There is growing evidence of a link between superoxide dismutase (SOD1) mutations and the formation of neurofilament depositions. It is suggested that abundant proteins with long half-lives in motor neurons such as neurofilament proteins would be favored targets of damage by SOD1 mutants. The chapter also elaborates the in vivo effects of neurofilament proteins in disease caused by SOD1 mutations." @default.
- W1183617902 created "2016-06-24" @default.
- W1183617902 creator A5091664460 @default.
- W1183617902 date "1998-01-01" @default.
- W1183617902 modified "2023-09-27" @default.
- W1183617902 title "Transgenic Mouse Models with Neurofilament-Induced Pathologies" @default.
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- W1183617902 doi "https://doi.org/10.1016/b978-012734610-6/50007-5" @default.
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