Polyglutamine-Expanded Ataxin-7 Promotes Non-Cell-Autonomous Purkinje Cell Degeneration and Displays Proteolytic Cleavage in Ataxic Transgenic Mice

Spinocerebellar ataxia (SCA) type 7 is an inherited neurodegenerative disorder caused by expansion of a polyglutamine tract within the ataxin-7 protein. To determine the molecular basis of polyglutamine neurotoxicity in this and other related disorders, we produced SCA7 transgenic mice that express...

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Pubblicato in:J Neurosci
Autori principali: Garden, Gwenn A., Libby, Randell T., Fu, Ying-Hui, Kinoshita, Yoshito, Huang, Jing, Possin, Daniel E., Smith, Annette C., Martinez, Refugio A., Fine, Gabriel C., Grote, Sara K., Ware, Carol B., Einum, David D., Morrison, Richard S., Ptacek, Louis J., Sopher, Bryce L., Spada, Albert R. La
Natura: Artigo
Lingua:Inglês
Pubblicazione: Society for Neuroscience 2002
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC6757746/
https://ncbi.nlm.nih.gov/pubmed/12077187
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.22-12-04897.2002
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