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Author Notes:

Correspondence: Dr. Anthony W.S. Chan; awchan@emory.edu; Phone: 404-712-8347; Fax: 404-727-5289

All experimental procedures described in this study were approved by Emory Environmental Health and Biosafety committee.

We thank Ms. Leslee Sinclair for her assistance in proofing and editing. Tanut Kunkanjanawan and Rangsan Pranpai are supported by the Royal Golden Jubilee PhD program of Thailand Research Fund.

Subjects:

Research Funding:

YNPRC is supported by the Office of Research and Infrastructure Program (ORIP)/OD P51OD11132.

This study is supported in part by grant awarded by the NINDS (NS084163) and the ORIP/NIH (OD010930; Transgenic Huntington’s Disease Monkey Resource) to AWSC.

Keywords:

  • Science & Technology
  • Life Sciences & Biomedicine
  • Physical Sciences
  • Biochemical Research Methods
  • Biotechnology & Applied Microbiology
  • Chemistry, Analytical
  • Biochemistry & Molecular Biology
  • Chemistry
  • Huntington's disease
  • monkey model
  • pluripotent stem cells
  • neural cells
  • drug screening
  • HUNTINGTONS-DISEASE
  • IN-VITRO
  • CELLULAR PHENOTYPES
  • RILUZOLE
  • MODEL
  • AGGREGATION
  • SURVIVAL
  • BLOCK
  • MICE

Induced Pluripotent HD Monkey Stem Cells Derived Neural Cells for Drug Discovery

Tools:

Journal Title:

Slas Discovery

Volume:

Volume 22, Number 6

Publisher:

, Pages 696-705

Type of Work:

Article | Post-print: After Peer Review

Abstract:

Huntington’s disease (HD) is a neurodegenerative disease caused by an expansion of CAG trinucleotide repeat (polyglutamine [polyQ]) in the huntingtin (HTT) gene, which leads to the formation of mutant HTT (mHTT) protein aggregates. In the nervous system, an accumulation of mHTT protein results in glutamate-mediated excitotoxicity, proteosome instability, and apoptosis. Although HD pathogenesis has been extensively studied, effective treatment of HD has yet to be developed. Therapeutic discovery research in HD has been reported using yeast, cells derived from transgenic animal models and HD patients, and induced pluripotent stem cells from patients. A transgenic nonhuman primate model of HD (HD monkey) shows neuropathological, behavioral, and molecular changes similar to an HD patient. In addition, neural progenitor cells (NPCs) derived from HD monkeys can be maintained in culture and differentiated to neural cells with distinct HD cellular phenotypes including the formation of mHTT aggregates, intranuclear inclusions, and increased susceptibility to oxidative stress. Here, we evaluated the potential application of HD monkey NPCs and neural cells as an in vitro model for HD drug discovery research.

Copyright information:

© 2016, © 2016 Society for Laboratory Automation and Screening.

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