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Ataxin-3 Protein and RNA Toxicity in Spinocerebellar Ataxia Type 3: Current Insights and Emerging Therapeutic Strategies

Ataxin-3 is a ubiquitously expressed deubiqutinating enzyme with important functions in the proteasomal protein degradation pathway and regulation of transcription. The C-terminus of the ataxin-3 protein contains a polyglutamine (PolyQ) region that, when mutationally expanded to over 52 glutamines,...

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Autori principali: Evers, Melvin M., Toonen, Lodewijk J. A., van Roon-Mom, Willeke M. C.
Natura: Artigo
Lingua:Inglês
Pubblicazione: Springer US 2013
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC4012159/
https://ncbi.nlm.nih.gov/pubmed/24293103
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s12035-013-8596-2
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