Nuclear inclusions of pathogenic ataxin-1 induce oxidative stress and perturb the protein synthesis machinery

Spinocerebellar ataxia type-1 (SCA1) is caused by an abnormally expanded polyglutamine (polyQ) tract in ataxin-1. These expansions are responsible for protein misfolding and self-assembly into intranuclear inclusion bodies (IIBs) that are somehow linked to neuronal death. However, owing to lack of a...

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Detalles Bibliográficos
Autores principales: Laidou, Stamatia, Alanis-Lobato, Gregorio, Pribyl, Jan, Raskó, Tamás, Tichy, Boris, Mikulasek, Kamil, Tsagiopoulou, Maria, Oppelt, Jan, Kastrinaki, Georgia, Lefaki, Maria, Singh, Manvendra, Zink, Annika, Chondrogianni, Niki, Psomopoulos, Fotis, Prigione, Alessandro, Ivics, Zoltán, Pospisilova, Sarka, Skladal, Petr, Izsvák, Zsuzsanna, Andrade-Navarro, Miguel A., Petrakis, Spyros
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2020
Materias:
Research Paper
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7058924/
https://www.ncbi.nlm.nih.gov/pubmed/32145456
http://dx.doi.org/10.1016/j.redox.2020.101458

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