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Dissecting Alzheimer disease in Down syndrome using mouse models

Choong, XY; Tosh, JL; Pulford, LJ; Fisher, EM; (2015) Dissecting Alzheimer disease in Down syndrome using mouse models. Frontiers in Behavioral Neuroscience , 9 , Article 268. 10.3389/fnbeh.2015.00268. Green open access

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Abstract

Down syndrome (DS) is a common genetic condition caused by the presence of three copies of chromosome 21 (trisomy 21). This greatly increases the risk of Alzheimer disease (AD), but although virtually all people with DS have AD neuropathology by 40 years of age, not all develop dementia. To dissect the genetic contribution of trisomy 21 to DS phenotypes including those relevant to AD, a range of DS mouse models has been generated which are trisomic for chromosome segments syntenic to human chromosome 21. Here, we consider key characteristics of human AD in DS (AD-DS), and our current state of knowledge on related phenotypes in AD and DS mouse models. We go on to review important features needed in future models of AD-DS, to understand this type of dementia and so highlight pathogenic mechanisms relevant to all populations at risk of AD.

Type: Article
Title: Dissecting Alzheimer disease in Down syndrome using mouse models
Location: Switzerland
Open access status: An open access version is available from UCL Discovery
DOI: 10.3389/fnbeh.2015.00268
Publisher version: http://dx.doi.org/10.3389/fnbeh.2015.00268
Language: English
Additional information: © 2015 Choong, Tosh, Pulford and Fisher. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
Keywords: APP, Alzheimer disease, Down syndrome, mouse models, trisomy 21
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 > Department of Neuromuscular Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/1473120
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