Cerebellar spreading depolarization mediates paroxysmal movement disorder

doi:10.1016/j.celrep.2021.109743

	Cerebellar spreading depolarization mediates paroxysmal movement disorder
	Lu, Bin 1; Lou, Sen-Sen 1,2; Xu, Ruo-Shui 1,2; Kong, De-Lun1,2,3 ; Wu, Rong-Jie1,2,3 ; Zhang, Jing 1; Zhuang, Ling 1,2; Wu, Xue-Mei1,2,3 ; He, Jun-Yan 1,2; Wu, Zhi-Ying 4,5; Xiong, Zhi-Qi 1,2,6
	2021-09-21
发表期刊	CELL REPORTS (IF:7.5[JCR-2023],8.5[5-Year])
ISSN	2211-1247
卷号	36 期号:12
DOI	10.1016/j.celrep.2021.109743
摘要	Paroxysmal kinesigenic dyskinesia (PKD) is the most common paroxysmal dyskinesia, characterized by recurrent episodes of involuntary movements provoked by sudden changes inmovement. Proline-rich transmembrane protein 2 (PRRT2) has been identified as the major causative gene for PKD. Here, we report that PRRT2 deficiency facilitates the induction of cerebellar spreading depolarization (SD) and inhibition of cerebellar SD prevents the occurrence of dyskinetic movements. Using Ca2+ imaging, we show that cerebellar SD depolarizes a large population of cerebellar granule cells and Purkinje cells in Prrt2-deficient mice. Electrophysiological recordings further reveal that cerebellar SD blocks Purkinje cell spiking and disturbs neuronal firing of the deep cerebellar nuclei (DCN). The resultant aberrant firing patterns in DCN are tightly, temporally coupled to dyskinetic episodes in Prrt2-deficient mice. Cumulatively, our findings uncover a pivotal role of cerebellar SD in paroxysmal dyskinesia, providing a potent target for treating PRRT2-related paroxysmal disorders.
收录类别	SCIE
语种	英语
WOS研究方向	Cell Biology
WOS类目	Cell Biology
WOS记录号	WOS:000698680800022
出版者	CELL PRESS
原始文献类型	Article
引用统计	正在获取...
文献类型	期刊论文
条目标识符	https://kms.shanghaitech.edu.cn/handle/2MSLDSTB/128329
专题	生命科学与技术学院_硕士生生命科学与技术学院_博士生
通讯作者	Xiong, Zhi-Qi
作者单位	1.Chinese Acad Sci, Ctr Excellence Brain Sci & Intelligence Technol, Inst Neurosci, State Key Lab Neurosci, Shanghai 200031, Peoples R China; 2.Univ Chinese Acad Sci, Beijing 100049, Peoples R China; 3.ShanghaiTech Univ, Sch Life Sci & Technol, Shanghai 201210, Peoples R China; 4.Zhejiang Univ, Sch Med, Dept Neurol, Affiliated Hosp 2, Hangzhou 310009, Zhejiang, Peoples R China; 5.Zhejiang Univ, Sch Med, Res Ctr Neurol, Affiliated Hosp 2, Hangzhou 310009, Zhejiang, Peoples R China; 6.Shanghai Ctr Brain Sci & Brain Inspired Intellige, Shanghai 201210, Peoples R China
推荐引用方式 GB/T 7714	Lu, Bin,Lou, Sen-Sen,Xu, Ruo-Shui,et al. Cerebellar spreading depolarization mediates paroxysmal movement disorder[J]. CELL REPORTS,2021,36(12).
APA	Lu, Bin.,Lou, Sen-Sen.,Xu, Ruo-Shui.,Kong, De-Lun.,Wu, Rong-Jie.,...&Xiong, Zhi-Qi.(2021).Cerebellar spreading depolarization mediates paroxysmal movement disorder.CELL REPORTS,36(12).
MLA	Lu, Bin,et al."Cerebellar spreading depolarization mediates paroxysmal movement disorder".CELL REPORTS 36.12(2021).