Silencing Mutant Ataxin-3 Rescues Motor Deficits and Neuropathology in Machado-Joseph Disease Transgenic Mice

Machado-Joseph disease (MJD) or spinocerebellar ataxia type 3 (SCA3) is an autosomal dominantly-inherited neurodegenerative disorder caused by the over-repetition of a CAG codon in the MJD1 gene. This expansion translates into a polyglutamine tract that confers a toxic gain-of-function to the mutant...

Descripción completa

Detalles Bibliográficos
Autores principales: Nóbrega, Clévio, Nascimento-Ferreira, Isabel, Onofre, Isabel, Albuquerque, David, Hirai, Hirokazu, Déglon, Nicole, de Almeida, Luís Pereira
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3551966/
https://www.ncbi.nlm.nih.gov/pubmed/23349684
http://dx.doi.org/10.1371/journal.pone.0052396

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3551966/
https://www.ncbi.nlm.nih.gov/pubmed/23349684
http://dx.doi.org/10.1371/journal.pone.0052396

Ejemplares similares