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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.
Chan, A. W. S., Jiang, J., & Chen, Y., et al. Progressive Cognitive Deficit, Motor Impairment and Striatal Pathology in a Transgenic Huntington Disease Monkey Model from Infancy to Adulthood. PLoS ONE, 10, e0122335. Copyright © 2015 Chan et al. 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 author and source are credited.
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VCU Neurology Publications
Timeline of progressive development of clinical features in HD monkeys.
S1_Table.docx (17 kB)
Definitions of behavioral measures in the ORDT.
S2_Fig.tif (4179 kB)
Coronal T1-weighted images of WT control monkey and rHD1 at 12, 24, 36, and 48 months of age.
S2_Table.docx (14 kB)
List of primers.
S3_Fig.tif (10536 kB)
Volumetric changes in HD brain observed in T1-weighted images of rHD1 and a control monkey at four years of age.
S4_Fig.tif (11793 kB)
Head circumference and body weight of HD monkeys.