唐氏综合症老龄化和阿尔茨海默病的神经病理学发展

Title:Aging in Down Syndrome and the Development of Alzheimer’s Disease Neuropathology

Volume: 13 Issue: 1

Author(s): Elizabeth Head, Ira T. Lott, Donna M. Wilcock and Cynthia A. Lemere

Affiliation:

关键词: Beta-amyloid

摘要: Chromosome 21, triplicated in Down Syndrome, contains several genes that are thought to play a critical role in the development of AD neuropathology. The overexpression of the gene for the amyloid precursor protein (APP), on chromosome 21, leads to early onset beta-amyloid (Aβ) plaques in DS. In addition to Aβ accumulation, middle-aged people with DS develop neurofibrillary tangles, cerebrovascular pathology, white matter pathology, oxidative damage, neuroinflammation and neuron loss. There is also evidence of potential compensatory responses in DS that benefit the brain and delay the onset of dementia after there is sufficient neuropathology for a diagnosis of AD. This review describes some of the existing literature and also highlights gaps in our knowledge regarding AD neuropathology in DS. It will be critical in the future to develop networked brain banks with standardized collection procedures to fully characterize the regional and temporal pathological events associated with aging in DS. As more information is acquired regarding AD evolution in DS, there will be opportunities to develop interventions that are age-appropriate to delay AD in DS.

Cite this article as:

Elizabeth Head, Ira T. Lott, Donna M. Wilcock and Cynthia A. Lemere , Aging in Down Syndrome and the Development of Alzheimer’s Disease Neuropathology, Current Alzheimer Research 2016; 13 (1) . https://dx.doi.org/10.2174/1567205012666151020114607