"Expression profiles of α-synuclein in cortical lesions of patients with FCD IIb and TSC, and FCD rats"

作者全名："Zhang, Li; Huang, Jun; Dai, Lu; Zhu, Gang; Yang, Xiao-Lin; He, Zeng; Li, Yu-Hong; Yang, Hui; Zhang, Chun-Qing; Shen, Kai-Feng; Liang, Ping"

作者地址："[Zhang, Li; Liang, Ping] Childrens Hosp Chongqing Med Univ, Natl Clin Res Ctr Child Hlth & Disorders, Dept Rehabil, Minist Educ Key Lab Child Dev & Disorders, Chongqing, Peoples R China; [Huang, Jun; Zhu, Gang; Yang, Xiao-Lin; He, Zeng; Yang, Hui; Zhang, Chun-Qing; Shen, Kai-Feng] Army Med Univ, Xinqiao Hosp, Epilepsy Res Ctr PLA, Dept Neurosurg, Chongqing, Peoples R China; [Dai, Lu; Yang, Hui] Chongqing Inst Brain & Intelligence, Guangyang Bay Lab, Chongqing, Peoples R China; [Li, Yu-Hong] Army Med Univ, Coll Basic Med, Dept Cell Biol, Chongqing, Peoples R China"

通信作者："Liang, P (通讯作者)，Childrens Hosp Chongqing Med Univ, Natl Clin Res Ctr Child Hlth & Disorders, Dept Rehabil, Minist Educ Key Lab Child Dev & Disorders, Chongqing, Peoples R China.; Shen, KF (通讯作者)，Army Med Univ, Xinqiao Hosp, Epilepsy Res Ctr PLA, Dept Neurosurg, Chongqing, Peoples R China."

来源：FRONTIERS IN NEUROLOGY

ESI学科分类：CLINICAL MEDICINE

WOS号：WOS:001106318800001

JCR分区：Q2

影响因子：2.7

年份：2023

卷号：14

期号：　

开始页：　

结束页：　

文献类型：Article

关键词：FCD IIb; TSC; FCD rats; alpha-syn; NMDAR

摘要："Background: Focal cortical dysplasia (FCD) IIb and tuberous sclerosis complex (TSC) are common causes of drug-resistant epilepsy in children. However, the etiologies related to the development of FCD IIb and TSC are not fully understood. alpha-synuclein (alpha-syn) is a member of synucleins family that plays crucial roles in modulating synaptic transmission in central nervous system. Here, we explored the expression profiles and potential pathogenic functions of alpha-syn in cortical lesions of epileptic patients with FCD IIb and TSC. Methods: Surgical specimens from epileptic patients with FCD IIb and TSC, as well as FCD rats generated by in utero X-ray-radiation were adopted in this study and studied with immunohistochemistry, immunofluorescence, western blotting, and co-immunoprecipitation etc. molecular biological techniques. Result: Our results showed that alpha-syn expression was reduced in FCD IIb and TSC lesions. Specifically, alpha-syn protein was intensely expressed in dysplastic neurons (DNs) and balloon cells (BCs) in FCD IIb lesions, whereas was barely detected in DNs and giant cells (GCs) of TSC lesions. Additionally, p-alpha-syn, the aggregated form of alpha-syn, was detected in DNs, BCs, GCs, and glia-like cells of FCD IIb and TSC lesions. We previous showed that the function of N-methyl-D-aspartate receptor (NMDAR) was enhanced in FCD rats generated by X-ray-radiation. Here, we found the interaction between alpha-syn and NMDAR subunits NMDAR2A, NMDAR2B were augmented in cortical lesions of FCD patients and FCD rats. Conclusion: These results suggested a potential role of alpha-syn in the pathogenesis of FCD IIb and TSC by interfering with NMDAR."

基金机构：National Natural Science Foundation of China [82171463]

基金资助正文：This work was supported by the grant from National Natural Science Foundation of China (no. 82171463).