Suppr超能文献

严重寡聚态 tau 毒性可逆转而无长期后遗症。

Severe oligomeric tau toxicity can be reversed without long-term sequelae.

机构信息

Institute of Medical Genetics and Pathology, University Hospital Basel, CH-4031 Basel, Switzerland.

Department of Neurology, University Hospital Basel, CH-4031 Basel, Switzerland.

出版信息

Brain. 2021 Apr 12;144(3):963-974. doi: 10.1093/brain/awaa445.

Abstract

Tau is a microtubule stabilizing protein that forms abnormal aggregates in many neurodegenerative disorders, including Alzheimer's disease. We have previously shown that co-expression of fragmented and full-length tau in P301SxTAU62on tau transgenic mice results in the formation of oligomeric tau species and causes severe paralysis. This paralysis is fully reversible once expression of the tau fragment is halted, even though P301S tau expression is maintained. Whereas various strategies to target tau aggregation have been developed, little is known about the long-term consequences of reverted tau toxicity. Therefore, we studied the long-term motor fitness of recovered, formerly paralysed P301SxTAU62on-off mice. To assess the seeding competence of oligomeric toxic tau species, we also inoculated ALZ17 mice with brainstem homogenates from paralysed P301SxTAU62on mice. Counter-intuitively, after recovery from paralysis due to oligomeric tau species expression, ageing P301SxTAU62on-off mice did not develop more motor impairment or tau pathology when compared to heterozygous P301S tau transgenic littermates. Thus, toxic tau species causing extensive neuronal dysfunction can be cleared without inducing seeding effects. Moreover, these toxic tau species also lack long-term tau seeding effects upon intrahippocampal inoculation into ALZ17 mice. In conclusion, tau species can be neurotoxic in the absence of seeding-competent tau aggregates, and mice can clear these tau forms permanently without tau seeding or spreading effects. These observations suggest that early targeting of non-fibrillar tau species may represent a therapeutically effective intervention in tauopathies. On the other hand, the absent seeding competence of early toxic tau species also warrants caution when using seeding-based tests for preclinical tauopathy diagnostics.

摘要

tau 是一种微管稳定蛋白,在包括阿尔茨海默病在内的许多神经退行性疾病中形成异常聚集物。我们之前已经表明,在 P301SxTAU62tau 转基因小鼠中共同表达片段化和全长 tau 会导致寡聚 tau 物种的形成,并导致严重的瘫痪。一旦停止表达 tau 片段,这种瘫痪完全可逆,尽管 P301S tau 的表达仍在维持。虽然已经开发了各种针对 tau 聚集的策略,但对于恢复的 tau 毒性的长期后果知之甚少。因此,我们研究了恢复的、以前瘫痪的 P301SxTAU62on-off 小鼠的长期运动适应能力。为了评估寡聚毒性 tau 物种的种子能力,我们还将来自瘫痪 P301SxTAU62on 小鼠的脑干匀浆接种到 ALZ17 小鼠中。反直觉的是,在由于寡聚 tau 物种表达而从瘫痪中恢复后,与杂合 P301S tau 转基因同窝仔鼠相比,年老的 P301SxTAU62on-off 小鼠没有出现更多的运动障碍或 tau 病理学。因此,引起广泛神经元功能障碍的毒性 tau 物种可以被清除而不会诱导种子效应。此外,这些毒性 tau 物种在向 ALZ17 小鼠海马内接种时也缺乏长期 tau 种子效应。总之,tau 物种在没有种子能力的 tau 聚集物的情况下也可能具有神经毒性,并且小鼠可以永久清除这些 tau 形式而没有 tau 种子或传播效应。这些观察结果表明,早期靶向非纤维状 tau 物种可能代表了 tau 病治疗上有效的干预措施。另一方面,早期毒性 tau 物种的缺乏种子能力也需要在使用基于种子的 tau 病前临床诊断测试时保持警惕。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6999/8041046/278b59e459bc/awaa445f1.jpg

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验