Severe neurological phenotypes of Q129 DRPLA transgenic mice serendipitously created by en masse expansion of CAG repeats in Q76 DRPLA mice

We herein provide a thorough description of new transgenic mouse models for dentatorubral–pallidoluysian atrophy (DRPLA) harboring a single copy of the full-length human mutant DRPLA gene with 76 and 129 CAG repeats. The Q129 mouse line was unexpectedly obtained by en masse expansion based on the so...

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Main Authors: Sato, Toshiya, Miura, Masami, Yamada, Mitsunori, Yoshida, Takayuki, Wood, Jonathan D., Yazawa, Ikuru, Masuda, Masao, Suzuki, Takeo, Shin, Ryong-Moon, Yau, Hau-Jie, Liu, Fu-Chin, Shimohata, Takayoshi, Onodera, Osamu, Ross, Christopher A., Katsuki, Motoya, Takahashi, Hitoshi, Kano, Masanobu, Aosaki, Toshihiko, Tsuji, Shoji
Format: Artigo
Jezik:Inglês
Izdano: Oxford University Press 2009
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Articles
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC2638829/
https://ncbi.nlm.nih.gov/pubmed/19039037
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddn403
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