A Novel Antisense CAG Repeat Transcript at JPH3 Locus Mediating Expanded Polyglutamine Protein Toxicity in Huntington’s Disease-Like 2 (HDL2) Mice

Huntington’s disease like-2 (HDL2) is a phenocopy of Huntington’s disease caused by CTG/CAG repeat expansion at the Junctophilin-3 (JPH3) locus. The mechanisms underlying HDL2 pathogenesis remain unclear. Here we developed a BAC transgenic mouse model of HDL2 (BAC-HDL2) that exhibits progressive mot...

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Detaylı Bibliyografya
Asıl Yazarlar: Wilburn, Brian, Rudnicki, Dobrila D., Zhao, Jing, Weitz, Tara Murphy, Cheng, Yin, Gu, Xiaofeng, Greiner, Erin, Park, Chang Sin, Wang, Nan, Sopher, Bryce L., La Spada, Albert R., Osmand, Alex, Margolis, Russell L., Sun, Yi E., Yang, X. William
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: 2011
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Article
Online Erişim:https://ncbi.nlm.nih.gov/pmc/articles/PMC3107122/
https://ncbi.nlm.nih.gov/pubmed/21555070
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.neuron.2011.03.021
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