A Novel SCA3 Knock-in Mouse Model Mimics the Human SCA3 Disease Phenotype Including Neuropathological, Behavioral, and Transcriptional Abnormalities Especially in Oligodendrocytes

Spinocerebellar ataxia type 3 is the most common autosomal dominant inherited ataxia worldwide, caused by a CAG repeat expansion in the Ataxin-3 gene resulting in a polyglutamine (polyQ)-expansion in the corresponding protein. The disease is characterized by neuropathological, phenotypical, and spec...

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Detalles Bibliográficos
Autores principales: Haas, Eva, Incebacak, Rana D., Hentrich, Thomas, Huridou, Chrisovalantou, Schmidt, Thorsten, Casadei, Nicolas, Maringer, Yacine, Bahl, Carola, Zimmermann, Frank, Mills, James D., Aronica, Eleonora, Riess, Olaf, Schulze-Hentrich, Julia M., Hübener-Schmid, Jeannette
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2021
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8786755/
https://www.ncbi.nlm.nih.gov/pubmed/34716557
http://dx.doi.org/10.1007/s12035-021-02610-8

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