摘要
唐氏综合征是最常见的非致死性遗传性疾病,在美国得出生人口大约1/700概率患有此症。DS的特点是完整的或节段性21染色体三体征,导致多变的智力障碍,进行性记忆力减退、随年龄的增长而加速的神经退行性改变。在过去的三十年, 由于合并症医学治疗的进步,DS人群增长了成倍的寿命,使得这类人群达到了发展成早发型阿尔茨海默病(AD)的年龄。在他们的第四个或第五个十年中,DS患者显露出AD认知和病理上的特征,目前缺乏对老年痴呆症成功的预防或治疗方案。与DS相关的AD(DS-AD)的深刻记忆障碍已与一些神经元数量的退化有关,但神经退行性病变的机制在很大程度上是未知的。最成功的DS动物模型的Ts65Dn老鼠,但一些新的模型也得到发展。在此综述里,我们讨论了最近的研究结果和的潜在的控制记忆力丧失的治疗选项以及DS模型老鼠的AD神经病理学。我们还回顾了年龄相关的神经病理学和最近的神经影像学研究新发现。确定合适的、模拟人类神经退行性疾病和DS记忆丧失的DS老鼠模型,在新的预防和治疗干预的研究里是有价值的,可能对准确描述参与神经退行性病变的基因-基因的相互作用以及特定的基因片段有帮助。
关键词: Cholinergic neurons
Current Alzheimer Research
Title:Cognitive Impairment, Neuroimaging, and Alzheimer Neuropathology in Mouse Models of Down Syndrome
Volume: 13 Issue: 1
Author(s): Eric D. Hamlett, Heather A. Boger, Aurélie Ledreux, Christy M. Kelley, Elliott J. Mufson and Maria F. Falangola, David N. Guilfoyle, Ralph A. Nixon, David Patterson, Nathan Duval and Ann-Charlotte E. Granholm
Affiliation:
关键词: Cholinergic neurons
摘要: Down syndrome (DS) is the most common non-lethal genetic condition that affects approximately 1 in 700 births in the United States of America. DS is characterized by complete or segmental chromosome 21 trisomy, which leads to variable intellectual disabilities, progressive memory loss, and accelerated neurodegeneration with age. During the last three decades, people with DS have experienced a doubling of life expectancy due to progress in treatment of medical comorbidities, which has allowed this population to reach the age when they develop early onset Alzheimer’s disease (AD). Individuals with DS develop cognitive and pathological hallmarks of AD in their fourth or fifth decade, and are currently lacking successful prevention or treatment options for dementia. The profound memory deficits associated with DS-related AD (DS-AD) have been associated with degeneration of several neuronal populations, but mechanisms of neurodegeneration are largely unexplored. The most successful animal model for DS is the Ts65Dn mouse, but several new models have also been developed. In the current review, we discuss recent findings and potential treatment options for the management of memory loss and AD neuropathology in DS mouse models. We also review agerelated neuropathology, and recent findings from neuroimaging studies. The validation of appropriate DS mouse models that mimic neurodegeneration and memory loss in humans with DS can be valuable in the study of novel preventative and treatment interventions, and may be helpful in pinpointing gene-gene interactions as well as specific gene segments involved in neurodegeneration.
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Eric D. Hamlett, Heather A. Boger, Aurélie Ledreux, Christy M. Kelley, Elliott J. Mufson and Maria F. Falangola, David N. Guilfoyle, Ralph A. Nixon, David Patterson, Nathan Duval and Ann-Charlotte E. Granholm , Cognitive Impairment, Neuroimaging, and Alzheimer Neuropathology in Mouse Models of Down Syndrome, Current Alzheimer Research 2016; 13 (1) . https://dx.doi.org/10.2174/1567205012666150921095505
DOI https://dx.doi.org/10.2174/1567205012666150921095505 |
Print ISSN 1567-2050 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5828 |
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