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Msh2 Acts in Medium-Spiny Striatal Neurons as an Enhancer of CAG Instability and Mutant Huntingtin Phenotypes in Huntington’s Disease Knock-In Mice

The CAG trinucleotide repeat mutation in the Huntington’s disease gene (HTT) exhibits age-dependent tissue-specific expansion that correlates with disease onset in patients, implicating somatic expansion as a disease modifier and potential therapeutic target. Somatic HTT CAG expansion is critically...

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Detalles Bibliográficos
Autores principales: Kovalenko, Marina, Dragileva, Ella, St. Claire, Jason, Gillis, Tammy, Guide, Jolene R., New, Jaclyn, Dong, Hualing, Kucherlapati, Raju, Kucherlapati, Melanie H., Ehrlich, Michelle E., Lee, Jong-Min, Wheeler, Vanessa C.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3436885/
https://www.ncbi.nlm.nih.gov/pubmed/22970194
http://dx.doi.org/10.1371/journal.pone.0044273