PMID- 38020594
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20231201
IS  - 1664-2295 (Print)
IS  - 1664-2295 (Electronic)
IS  - 1664-2295 (Linking)
VI  - 14
DP  - 2023
TI  - Expression profiles of alpha-synuclein in cortical lesions of patients with FCD IIb 
      and TSC, and FCD rats.
PG  - 1255097
LID - 10.3389/fneur.2023.1255097 [doi]
LID - 1255097
AB  - 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.
CI  - Copyright (c) 2023 Zhang, Huang, Dai, Zhu, Yang, He, Li, Yang, Zhang, Shen and 
      Liang.
FAU - Zhang, Li
AU  - Zhang L
AD  - Department of Neurosurgery, Children's Hospital of Chongqing Medical University, 
      National Clinical Research Center for Child Health and Disorders, Ministry of 
      Education Key Laboratory of Child Development and Disorders, Chongqing Key 
      Laboratory of Pediatrics, Chongqing, China.
FAU - Huang, Jun
AU  - Huang J
AD  - Department of Neurosurgery, Epilepsy Research Center of PLA, Xinqiao Hospital, 
      Army Medical University, Chongqing, China.
FAU - Dai, Lu
AU  - Dai L
AD  - Chongqing Institute for Brain and Intelligence, Guang Yang Bay Laboratory, 
      Chongqing, China.
FAU - Zhu, Gang
AU  - Zhu G
AD  - Department of Neurosurgery, Epilepsy Research Center of PLA, Xinqiao Hospital, 
      Army Medical University, Chongqing, China.
FAU - Yang, Xiao-Lin
AU  - Yang XL
AD  - Department of Neurosurgery, Epilepsy Research Center of PLA, Xinqiao Hospital, 
      Army Medical University, Chongqing, China.
FAU - He, Zeng
AU  - He Z
AD  - Department of Neurosurgery, Epilepsy Research Center of PLA, Xinqiao Hospital, 
      Army Medical University, Chongqing, China.
FAU - Li, Yu-Hong
AU  - Li YH
AD  - Department of Cell Biology, Basic Medical College, Army Medical University, 
      Chongqing, China.
FAU - Yang, Hui
AU  - Yang H
AD  - Department of Neurosurgery, Epilepsy Research Center of PLA, Xinqiao Hospital, 
      Army Medical University, Chongqing, China.
AD  - Chongqing Institute for Brain and Intelligence, Guang Yang Bay Laboratory, 
      Chongqing, China.
FAU - Zhang, Chun-Qing
AU  - Zhang CQ
AD  - Department of Neurosurgery, Epilepsy Research Center of PLA, Xinqiao Hospital, 
      Army Medical University, Chongqing, China.
FAU - Shen, Kai-Feng
AU  - Shen KF
AD  - Department of Neurosurgery, Epilepsy Research Center of PLA, Xinqiao Hospital, 
      Army Medical University, Chongqing, China.
FAU - Liang, Ping
AU  - Liang P
AD  - Department of Neurosurgery, Children's Hospital of Chongqing Medical University, 
      National Clinical Research Center for Child Health and Disorders, Ministry of 
      Education Key Laboratory of Child Development and Disorders, Chongqing Key 
      Laboratory of Pediatrics, Chongqing, China.
LA  - eng
PT  - Journal Article
DEP - 20231107
PL  - Switzerland
TA  - Front Neurol
JT  - Frontiers in neurology
JID - 101546899
PMC - PMC10662349
OTO - NOTNLM
OT  - FCD IIb
OT  - FCD rats
OT  - NMDAR
OT  - TSC
OT  - alpha-syn
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2023/11/29 18:42
MHDA- 2023/11/29 18:43
PMCR- 2023/11/07
CRDT- 2023/11/29 14:47
PHST- 2023/07/08 00:00 [received]
PHST- 2023/10/06 00:00 [accepted]
PHST- 2023/11/29 18:43 [medline]
PHST- 2023/11/29 18:42 [pubmed]
PHST- 2023/11/29 14:47 [entrez]
PHST- 2023/11/07 00:00 [pmc-release]
AID - 10.3389/fneur.2023.1255097 [doi]
PST - epublish
SO  - Front Neurol. 2023 Nov 7;14:1255097. doi: 10.3389/fneur.2023.1255097. eCollection 
      2023.