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Plasma PolyQ-ATXN3 Levels Associate With Cerebellar Degeneration and Behavioral Abnormalities in a New AAV-Based SCA3 Mouse Model
Spinocerebellar ataxia type 3 (SCA3) is a dominantly inherited cerebellar ataxia caused by the expansion of a polyglutamine (polyQ) repeat in the gene encoding ATXN3. The polyQ expansion induces protein inclusion formation in the neurons of patients and results in neuronal degeneration in the cerebe...
Autores principales: | Jansen-West, Karen, Todd, Tiffany W., Daughrity, Lillian M., Yue, Mei, Tong, Jimei, Carlomagno, Yari, Del Rosso, Giulia, Kurti, Aishe, Jones, Caroline Y., Dunmore, Judith A., Castanedes-Casey, Monica, Dickson, Dennis W., Wszolek, Zbigniew K., Fryer, John D., Petrucelli, Leonard, Prudencio, Mercedes |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8977414/ https://www.ncbi.nlm.nih.gov/pubmed/35386195 http://dx.doi.org/10.3389/fcell.2022.863089 |
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