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Author Notes:

Email Address: awchan@emory.edu

Conceived and designed the experiments: AWSC SL XL JB. Performed the experiments: JJ YC CL MSP TC SM TR. Analyzed the data: AWSC JJ YC CL MSP YH TR HM RV JB.

Contributed reagents/materials/analysis tools: AWSC YS XZ JB. Wrote the paper: AWSC JJ YC CL MSP YH CMT SMZ JB.

We thank the Yerkes National Primate Research Center (YNPRC) veterinarians, animal care staff, and imaging center staff for providing outstanding services, and Cheryl Timms Strauss for editorial assistance.

Special thanks to the veterinarian staff, primate enrichment team, and animal care personnel for providing superior medical and daily care to HD monkeys as disease progressed.

We thank Susan Jenkins for assistance in the neuropathological study.

We also thank current and past members of the Chan team who contributed to the development of the HD monkey model.

The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.

The authors have declared that no competing interests exist.

Subjects:

Research Funding:

AWSC: NIH/OD010930. YNPRC: NIH/P51OD11132. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript. Go to:

Progressive Cognitive Deficit, Motor Impairment and Striatal Pathology in a Transgenic Huntington Disease Monkey Model from Infancy to Adulthood

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Journal Title:

PLoS ONE

Volume:

Volume 10, Number 5

Publisher:

, Pages e0122335-e0122335

Type of Work:

Article | Final Publisher PDF

Abstract:

One of the roadblocks to developing effective therapeutics for Huntington disease (HD) is the lack of animal models that develop progressive clinical traits comparable to those seen in patients. Here we report a longitudinal study that encompasses cognitive and motor assessment, and neuroimaging of a group of transgenic HD and control monkeys from infancy to adulthood. Along with progressive cognitive and motor impairment, neuroimaging revealed a progressive reduction in striatal volume. Magnetic resonance spectroscopy at 48 months of age revealed a decrease of N-acetylaspartate (NAA), further suggesting neuronal damage/loss in the striatum. Postmortem neuropathological analyses revealed significant neuronal loss in the striatum. Our results indicate that HD monkeys share similar disease patterns with HD patients, making them potentially suitable as a preclinical HD animal model.

Copyright information:

© 2015 Chan et al.

This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits distribution of derivative works, making multiple copies, distribution, public display, and publicly performance, provided the original work is properly cited. This license requires credit be given to copyright holder and/or author, copyright and license notices be kept intact.

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