作者
Janelle Drouin‐Ouellet, Stephen J Sawiak, Giulia Cisbani, Marie Lagacé, Wei‐Li Kuan, Martine Saint‐Pierre, Richard J Dury, Wael Alata, Isabelle St‐Amour, Sarah L Mason, Frédéric Calon, Steve Lacroix, Penny A Gowland, Susan T Francis, Roger A Barker, Francesca Cicchetti
发表日期
2015/8
期刊
Annals of neurology
卷号
78
期号
2
页码范围
160-177
简介
Objective
Although the underlying cause of Huntington's disease (HD) is well established, the actual pathophysiological processes involved remain to be fully elucidated. In other proteinopathies such as Alzheimer's and Parkinson's diseases, there is evidence for impairments of the cerebral vasculature as well as the blood–brain barrier (BBB), which have been suggested to contribute to their pathophysiology. We investigated whether similar changes are also present in HD.
Methods
We used 3‐ and 7‐Tesla magnetic resonance imaging as well as postmortem tissue analyses to assess blood vessel impairments in HD patients. Our findings were further investigated in the R6/2 mouse model using in situ cerebral perfusion, histological analysis, Western blotting, as well as transmission and scanning electron microscopy.
Results
We found mutant huntingtin protein (mHtt) aggregates to be present in all major …
学术搜索中的文章
J Drouin‐Ouellet, SJ Sawiak, G Cisbani, M Lagacé… - Annals of neurology, 2015
