Suppr超能文献

小脑对肌张力障碍的影响:揭示浦肯野细胞和小脑核的作用

Cerebellar contributions to dystonia: unraveling the role of Purkinje cells and cerebellar nuclei.

作者信息

Jackson Nichelle N, Stagray Jacob A, Snell Heather D

机构信息

Department of Neuroscience, Yale School of Medicine, New Haven, CT, United States.

Wu Tsai Institute, Yale School of Medicine, New Haven, CT, United States.

出版信息

Dystonia. 2025;4. doi: 10.3389/dyst.2025.14006. Epub 2025 Feb 16.

Abstract

Dystonias are a group of neurodegenerative disorders that result in altered physiology associated with motor movements. Both the basal ganglia and the cerebellum, brain regions involved in motor learning, sensory perception integration, and reward, have been implicated in the pathology of dystonia, but the cellular and subcellular mechanisms remain diverse and for some forms of dystonia, elusive. The goal of the current review is to summarize recent evidence of cerebellar involvement in different subtypes of dystonia with a focus on Purkinje cell (PC) and cerebellar nuclei (CN) dysfunction, to find commonalities in the pathology that could lay the groundwork for the future development of therapeutics for patients with dystonia. Here we will briefly discuss the physical and functional connections between the basal ganglia and the cerebellum and how these connections could contribute to dystonic symptoms. We proceed to use human and animal model data to discuss the contributions of cerebellar cell types to specific dystonias and movement disorders where dystonia is a secondary symptom. Ultimately, we suggest PC and CN irregularity could be a locus for dystonia through impaired calcium dynamics.

摘要

肌张力障碍是一组神经退行性疾病,会导致与运动相关的生理改变。基底神经节和小脑这两个参与运动学习、感觉感知整合及奖赏的脑区,都与肌张力障碍的病理机制有关,但细胞和亚细胞机制仍然多种多样,对于某些形式的肌张力障碍来说,还难以捉摸。本综述的目的是总结小脑参与不同亚型肌张力障碍的最新证据,重点关注浦肯野细胞(PC)和小脑核(CN)功能障碍,以找出病理过程中的共性,为未来开发肌张力障碍患者的治疗方法奠定基础。在此,我们将简要讨论基底神经节和小脑之间的物理和功能联系,以及这些联系如何导致肌张力障碍症状。我们将利用人类和动物模型数据,讨论小脑细胞类型对特定肌张力障碍和以肌张力障碍为次要症状的运动障碍的影响。最终,我们认为PC和CN的异常可能是由于钙动力学受损而成为肌张力障碍的一个发病部位。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b56c/11925549/836c4337f695/nihms-2058682-f0001.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