Osmand, AP;
Bichell, TJ;
Bowman, AB;
Bates, GP;
(2016)
Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease.
Journal of Huntington's Disease
, 5
(4)
pp. 343-346.
10.3233/JHD-160217.
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Abstract
The role of aggregate formation in the pathophysiology of Huntington’s disease (HD) remains uncertain. However, the temporal appearance of aggregates tends to correlate with the onset of symptoms and the numbers of neuropil aggregates correlate with the progression of clinical disease. Using highly sensitive immunohistochemical methods we have detected the appearance of diffuse aggregates during embryonic development in the R6/2 and YAC128 mouse models of HD. These are initially seen in developing axonal tracts and appear to spread throughout the cerebrum in the early neonate.
| Type: | Article |
|---|---|
| Title: | Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.3233/JHD-160217 |
| Publisher version: | http://dx.doi.org/10.3233/JHD-160217 |
| Language: | English |
| Additional information: | © 2016. IOS Press and the authors. All rights reserved This article is published online with Open Access and distributed under the terms of the Creative Commons Attribution Non-Commercial License (CC BY- NC 4.0). |
| Keywords: | Huntington’s disease, mouse models, aggregation, development |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neurodegenerative Diseases |
| URI: | https://discovery.ucl.ac.uk/id/eprint/1537506 |
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