A two years longitudinal study of a transgenic Huntington disease monkey
| Autor: | Heather Banta, Katie Nelson, Sean P. Moran, Heidi Engelhardt, Yan Xu, Jannet Kocerha, Anthony W.S. Chan, Katherine Larkin, Adam Neumann, Tim Chi, Jie Jiang, Jinjing Yang, Xiaodong Zhang, Claudia M. Testa, Dongming Zhao, Francois Villinger, Hui Mao, Stuart M. Zola, Jocelyne Bachevalier, Haiying Cheng, Tayeb Rahim, Chun-Xia Li |
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| Jazyk: | angličtina |
| Předmět: |
Male
Pathology medicine.medical_specialty Longitudinal study Aging Huntingtin Hippocampus Nerve Tissue Proteins Striatum Hippocampal formation Animals Genetically Modified 03 medical and health sciences Cellular and Molecular Neuroscience 0302 clinical medicine biology.animal medicine Animals Humans Primate Tissue Distribution Longitudinal Studies Cognitive decline 030304 developmental biology Recognition memory 0303 health sciences Huntingtin Protein biology General Neuroscience Brain Organ Size Macaca mulatta 3. Good health Disease Models Animal Huntington Disease Neuroscience 030217 neurology & neurosurgery Research Article |
| Zdroj: | BMC Neuroscience |
| ISSN: | 1471-2202 |
| DOI: | 10.1186/1471-2202-15-36 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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