Peripheral androgen receptor gene suppression rescues disease in mouse models of spinal and bulbar muscular atrophy.

@article{Lieberman2014PeripheralAR,
  title={Peripheral androgen receptor gene suppression rescues disease in mouse models of spinal and bulbar muscular atrophy.},
  author={Andrew P. Lieberman and Zhigang Yu and Sue Murray and Raechel Q Peralta and Audrey K Low and Shuling Guo and Xing Xian Yu and Constanza J. Cortes and C. F. Bennett and Brett P. Monia and Albert R La Spada and Gene H Hung},
  journal={Cell reports},
  year={2014},
  volume={7 3},
  pages={774-84}
}
Spinal and bulbar muscular atrophy (SBMA) is caused by the polyglutamine androgen receptor (polyQ-AR), a protein expressed by both lower motor neurons and skeletal muscle. Although viewed as a motor neuronopathy, data from patients and mouse models suggest that muscle contributes to disease pathogenesis. Here, we tested this hypothesis using AR113Q knockin and human bacterial artificial chromosome/clone (BAC) transgenic mice that express the full-length polyQ-AR and display androgen-dependent… CONTINUE READING
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