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Striatal Mutant Huntingtin Protein Levels Decline with Age in Homozygous Huntington’s Disease Knock-In Mouse Models

BACKGROUND: Huntington’s disease (HD) is a progressive neurodegenerative disorder associated with aging, caused by an expanded polyglutamine (polyQ) repeat within the Huntingtin (HTT) protein. In HD, degeneration of the striatum and atrophy of the cortex are observed while cerebellum is less affecte...

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Detalles Bibliográficos
Autores principales: Franich, Nicholas R., Basso, Manuela, André, Emily A., Ochaba, Joseph, Kumar, Amit, Thein, Soe, Fote, Gianna, Kachemov, Marketta, Lau, Alice L., Yeung, Sylvia Y., Osmand, Alexander, Zeitlin, Scott O., Ratan, Rajiv R., Thompson, Leslie M., Steffan, Joan S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: IOS Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6002862/
https://www.ncbi.nlm.nih.gov/pubmed/29843246
http://dx.doi.org/10.3233/JHD-170274