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A Two Years Longitudinal Study of a Transgenic Huntington Disease Monkey
BMC Neuroscience (2014)
  • Anthony W. S. Chan, Emory University
  • Yan Xu
  • Jie Jiang, Emory University
  • Tayeb Rahim, Emory University
  • Jannet Kocerha, Georgia Southern University
  • Dongming Zhao, Emory University
  • Tim Chi, Emory University
  • Heidi Engelhardt, Emory University
  • Sean Moran, Emory University
  • Katherine Larkin, Emory University
  • Adam Neumann, Emory University
  • Haiying Cheng, Emory University
  • Chunxia Li, Emory University
  • Katie Nelson, Emory University
  • Heather Banta, Emory University
  • Stuart M. Zola, Emory University
  • Francois Villinger, Emory University
  • Jinjing Yang, Emory University
  • Claudia M. Testa, Emory University
  • Hui Mao, Emory University
  • Xiaodong Zhang, Emory University
  • Jocelyne Bachevalier, Emory University

Background: A two-year longitudinal study composed of morphometric MRI measures and cognitive behavioral evaluation was performed on a transgenic Huntington’s disease (HD) monkey. rHD1, a transgenic HD monkey expressing exon 1 of the human gene encoding huntingtin (HTT) with 29 CAG repeats regulated by a human polyubiquitin C promoter was used together with four age-matched wild-type control monkeys. This is the first study on a primate model of human HD based on longitudinal clinical measurements.

Results: Changes in striatal and hippocampal volumes in rHD1 were observed with progressive impairment in motor functions and cognitive decline, including deficits in learning stimulus-reward associations, recognition memory and spatial memory. The results demonstrate a progressive cognitive decline and morphometric changes in the striatum and hippocampus in a transgenic HD monkey.

Conclusions: This is the first study on a primate model of human HD based on longitudinal clinical measurements. While this study is based a single HD monkey, an ongoing longitudinal study with additional HD monkeys will be important for the confirmation of our findings. A nonhuman primate model of HD could complement other animal models of HD to better understand the pathogenesis of HD and future development of diagnostics and therapeutics through longitudinal assessment.

  • Huntington disease,
  • HD,
  • monkey
Publication Date
Publisher Statement
© 2014 Chan et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver applies to the data made available in this article, unless otherwise stated. Article originally published and obtained from BMC Neuroscience.
Citation Information
Anthony W. S. Chan, Yan Xu, Jie Jiang, Tayeb Rahim, Jannet Kocerha, Dongming Zhao, Tim Chi, Heidi Engelhardt, Sean Moran, Katherine Larkin, Adam Neumann, Haiying Cheng, Chunxia Li, Katie Nelson, Heather Banta, Stuart M. Zola, Francois Villinger, Jinjing Yang, Claudia M. Testa, Hui Mao, Xiaodong Zhang, and Jocelyne Bachevalier. "A Two Years Longitudinal Study of a Transgenic Huntington Disease Monkey" BMC Neuroscience 15.36 (2014).
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