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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,...
Autores principales: | Evers, Melvin M., Toonen, Lodewijk J. A., van Roon-Mom, Willeke M. C. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer US
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4012159/ https://www.ncbi.nlm.nih.gov/pubmed/24293103 http://dx.doi.org/10.1007/s12035-013-8596-2 |
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