Systemic administration of Congo red does not improve motor or cognitive function in R6/2 mice
Systemic administration of Congo red does not improve motor or cognitive function in R6/2 mice
复制标题
全身给予刚果红并不能改善 R6/2 小鼠的运动或认知功能
DOI：
10.1016/j.nbd.2006.09.015
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发表时间：
2007
影响因子：
6.1
通讯作者：
A. Morton
中科院分区：
文献类型：
--
作者：
N. Wood;Patrick N Pallier;J. Wanderer;A. Morton
亨廷顿病(HD)是一种进行性神经退行性疾病，目前尚无治疗方法。在症状出现之前，在人类和R6/2小鼠的神经元中发现了异常的蛋白质聚集体(包涵体)。有人提出，HD的进展可以通过破坏聚集过程来减缓或防止。与此一致的是，据报道，用刚果红对R6/2小鼠进行系统治疗后，纹状体内含物的数量减少，运动症状和存活率得到改善[Sanchez，I.，Mahlke，C.，袁，J.，2003。寡聚在扩张型聚谷氨酰胺神经退行性疾病中的关键作用。大自然421,373-379]。在这里，我们试图复制这项研究。我们扩大了实验范围，包括测量刚果红对R6/2小鼠认知功能的影响。刚果红治疗未能改善R6/2小鼠的运动或认知障碍。我们认为这是由于刚果红不能通过血脑屏障造成的。由于刚果红不能改善HD的一个关键特征--行为恶化，因此它不太可能用作HD的治疗方法。
Huntington’s disease (HD) is a progressive neurodegenerative disorder for which there is no treatment. Prior to the onset of symptoms, abnormal protein aggregates (inclusions) are found in neurons in humans and R6/2 mice. It has been suggested that the progression of HD can be slowed or prevented by disruption of the aggregation process. In agreement with this, it has been reported that systemic treatment of R6/2 mice with Congo red caused a reduction in numbers of striatal inclusions and an improvement in motor symptoms and survival [Sanchez, I., Mahlke, C., Yuan, J., 2003. Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders. Nature 421, 373–379]. Here we attempted to replicate this study. We extended the experiment to include measurement of the effects of Congo red on cognitive function in R6/2 mice. Congo red treatment failed to ameliorate either motor or cognitive deficits in R6/2 mice. We suggest that this is due to the inability of Congo red to cross the blood–brain barrier. Since it does not improve the behavioural deterioration that is a key feature of HD, Congo red is unlikely to be useful as a therapy for HD.
影响因子：
56.9
作者：
DiFiglia, M;Sapp, E;Aronin, N
通讯作者：
Aronin, N