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Intranuclear Aggregation of Mutant FUS/TLS as a Molecular Pathomechanism of Amyotrophic Lateral Sclerosis

Dominant mutations in FUS/TLS cause a familial form of amyotrophic lateral sclerosis (fALS), where abnormal accumulation of mutant FUS proteins in cytoplasm has been observed as a major pathological change. Many of pathogenic mutations have been shown to deteriorate the nuclear localization signal i...

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Autors principals: Nomura, Takao, Watanabe, Shoji, Kaneko, Kumi, Yamanaka, Koji, Nukina, Nobuyuki, Furukawa, Yoshiaki
Format: Artigo
Idioma:Inglês
Publicat: American Society for Biochemistry and Molecular Biology 2014
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC3887186/
https://ncbi.nlm.nih.gov/pubmed/24280224
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M113.516492
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