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Mutant Huntingtin Gene-Dose Impacts on Aggregate Deposition, DARPP32 Expression and Neuroinflammation in HdhQ150 Mice

Huntington's disease (HD) is an autosomal dominant, progressive and fatal neurological disorder caused by an expansion of CAG repeats in exon-1 of the huntingtin gene. The encoded poly-glutamine stretch renders mutant huntingtin prone to aggregation. HdhQ150 mice genocopy a pathogenic repeat (∼...

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Detalles Bibliográficos
Autores principales: Young, Douglas, Mayer, Franziska, Vidotto, Nella, Schweizer, Tatjana, Berth, Ramon, Abramowski, Dorothee, Shimshek, Derya R., van der Putten, P. Herman, Schmid, Peter
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3781050/
https://www.ncbi.nlm.nih.gov/pubmed/24086450
http://dx.doi.org/10.1371/journal.pone.0075108