Cargando…
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...
Autores principales: | 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 |
---|---|
Formato: | Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2009
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2638829/ https://www.ncbi.nlm.nih.gov/pubmed/19039037 http://dx.doi.org/10.1093/hmg/ddn403 |
Ejemplares similares
-
Case report of optic atrophy in Dentatorubropallidoluysian Atrophy (DRPLA)
por: Silver, Michael R., et al.
Publicado: (2015) -
Machine Learning Approach for the Prediction of Age-Specific Probability of SCA3 and DRPLA by Survival Curve Analysis
por: Hatano, Yuya, et al.
Publicado: (2023) -
Efficacy of perampanel for controlling seizures and improving neurological dysfunction in a patient with dentatorubral-pallidoluysian atrophy (DRPLA)
por: Shiraishi, Hideaki, et al.
Publicado: (2017) -
DRPLA: understanding the natural history and developing biomarkers to accelerate therapeutic trials in a globally rare repeat expansion disorder
por: Chaudhry, Aiysha, et al.
Publicado: (2020) -
Correction to: DRPLA: understanding the natural history and developing biomarkers to accelerate therapeutic trials in a globally rare repeat expansion disorder
por: Chaudhry, Aiysha, et al.
Publicado: (2021)