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Absence of neurofilaments reduces the selective vulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant

Mutations in superoxide dismutase 1 (SOD1), the only proven cause of amyotrophic lateral sclerosis (ALS), provoke disease through an unidentified toxic property. Neurofilament aggregates are pathologic hallmarks of both sporadic and SOD1-mediated familial ALS. By deleting NF-L, the major neurofilame...

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Veröffentlicht in:Proc Natl Acad Sci U S A
Hauptverfasser: Williamson, Toni L., Bruijn, Lucie I., Zhu, Qinzhang, Anderson, Karen L., Anderson, Scott D., Julien, Jean-Pierre, Cleveland, Don W.
Format: Artigo
Sprache:Inglês
Veröffentlicht: National Academy of Sciences 1998
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Online-Zugang:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC21390/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/9689132/
https://ncbi.nlm.nih.govhttps://doi.org/10.1073/pnas.95.16.9631
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