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Published in: Journal of Neural Transmission 3/2018

01-03-2018 | Translational Neurosciences - Review Article

Progress in developing transgenic monkey model for Huntington’s disease

Authors: Brooke R. Snyder, Anthony W. S. Chan

Published in: Journal of Neural Transmission | Issue 3/2018

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Abstract

Huntington’s disease (HD) is a complex neurodegenerative disorder that has no cure. Although treatments can often be given to relieve symptoms, the neuropathology associated with HD cannot be stopped or reversed. HD is characterized by degeneration of the striatum and associated pathways that leads to impairment in motor and cognitive functions as well as psychiatric disturbances. Although cell and rodent models for HD exist, longitudinal study in a transgenic HD nonhuman primate (i.e., rhesus macaque; HD monkeys) shows high similarity in its progression with human patients. Progressive brain atrophy and changes in white matter integrity examined by magnetic resonance imaging are coherent with the decline in cognitive behaviors related to corticostriatal functions and neuropathology. HD monkeys also express higher anxiety and irritability/aggression similar to human HD patients that other model systems have not yet replicated. While a comparative model approach is critical for advancing our understanding of HD pathogenesis, HD monkeys could provide a unique platform for preclinical studies and long-term assessment of translatable outcome measures. This review summarizes the progress in the development of the transgenic HD monkey model and the opportunities for advancing HD preclinical research.
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Metadata
Title
Progress in developing transgenic monkey model for Huntington’s disease
Authors
Brooke R. Snyder
Anthony W. S. Chan
Publication date
01-03-2018
Publisher
Springer Vienna
Published in
Journal of Neural Transmission / Issue 3/2018
Print ISSN: 0300-9564
Electronic ISSN: 1435-1463
DOI
https://doi.org/10.1007/s00702-017-1803-y

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