Pumilio1 Haploinsufficiency Leads to SCA1-like Neurodegeneration by Increasing Wild-Type Ataxin1 Levels

Spinocerebellar ataxia type 1 (SCA1) is a paradigmatic neurodegenerative proteinopathy, in which a mutant protein (in this case, ATAXIN1) accumulates in neurons and exerts toxicity; in SCA1 this process causes progressive deterioration of motor coordination. Seeking to understand how post-translatio...

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Publicat a:Cell
Autors principals: Gennarino, Vincenzo A., Singh, Ravi K., White, Joshua J., De Maio, Antonia, Han, Kihoon, Kim, Ji-Yoen, Jafar-Nejad, Paymaan, di Ronza, Alberto, Kang, Hyojin, Sayegh, Layal S., Cooper, Thomas A., Orr, Harry T., Sillitoe, Roy V., Zoghbi, Huda Y.
Format: Artigo
Idioma:Inglês
Publicat: 2015
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Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4383046/
https://ncbi.nlm.nih.gov/pubmed/25768905
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.cell.2015.02.012
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