Title |
Chronic Progressive Neurodegeneration in a Transgenic Mouse Model of Prion Disease
|
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Published in |
Frontiers in Neuroscience, November 2016
|
DOI | 10.3389/fnins.2016.00510 |
Pubmed ID | |
Authors |
Nina Fainstein, Dvir Dori, Kati Frid, Alexa T. Fritz, Ilona Shapiro, Ruth Gabizon, Tamir Ben-Hur |
Abstract |
Neurodegenerative diseases present pathologically with progressive structural destruction of neurons and accumulation of mis-folded proteins specific for each condition leading to brain atrophy and functional disability. Many animal models exert deposition of pathogenic proteins without an accompanying neurodegeneration pattern. The lack of a comprehensive model hinders efforts to develop treatment. We performed longitudinal quantification of cellular, neuronal and synaptic density, as well as of neurogenesis in brains of mice mimicking for genetic Creutzfeldt-Jacob disease as compared to age-matched wild-type mice. Mice exhibited a neurodegenerative process of progressive reduction in cortical neurons and synapses starting at age of 4-6 months, in accord with neurologic disability. This was accompanied by significant decrease in subventricular/subependymal zone neurogenesis. Although increased hippocampal neurogenesis was detected in mice, a neurodegenerative process of CA1 and CA3 regions associated with impaired hippocampal-dependent memory function was observed. In conclusion, mice exhibit pathological neurodegeneration concomitant with neurological disease progression, indicating these mice can serve as a model for neurodegenerative diseases. |
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Geographical breakdown
Country | Count | As % |
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Unknown | 16 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
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Student > Bachelor | 4 | 25% |
Student > Master | 3 | 19% |
Professor | 1 | 6% |
Researcher | 1 | 6% |
Librarian | 1 | 6% |
Other | 0 | 0% |
Unknown | 6 | 38% |
Readers by discipline | Count | As % |
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Neuroscience | 4 | 25% |
Agricultural and Biological Sciences | 3 | 19% |
Biochemistry, Genetics and Molecular Biology | 2 | 13% |
Psychology | 1 | 6% |
Unknown | 6 | 38% |