Altered expression of the Plexin-B2 system in tuberous sclerosis complex and focal cortical dysplasia IIb lesions

• Lu Dai ^[3] ; Jun Huang ^[3] ; Kai-feng Shen ^[3] ; Xiao-lin Yang ^[3] ; Gang Zhu ^[3] ; Li Zhang ^[1] ; Zhong-ke Wang ^[4] ; Shi-yong Liu ^[3] ; Xiang Liao ^[5] ; Sen-lin Xu ^[2] ; Hui Yang ^[3] ; Xing-yi Li ^[5] ; Chun-qing Zhang ^[3]
  1. [1] Children's Hospital of Chongqing Medical University

     Children's Hospital of Chongqing Medical University

     China

     
  2. [2] Southwest Hospital

     Southwest Hospital

     China

     
  3. [3] Epilepsy Research Center of PLA, Xinqiao Hospital, Army Medical University, PR China
  4. [4] Armed Police Hospital of Chongqing,PR China
  5. [5] School of Medicine, Chongqing University,PR China

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• Localización: Histology and histopathology: cellular and molecular biology, ISSN-e 1699-5848, ISSN 0213-3911, Vol. 39, Nº. 9, 2024, págs. 1179-1195
• Idioma: inglés
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• Resumen

  • Tuberous sclerosis complex (TSC) and focal cortical dysplasia (FCD) type IIb are the predominant causes of drug-refractory epilepsy in children.

    Dysmorphic neurons (DNs), giant cells (GCs), and balloon cells (BCs) are the most typical pathogenic profiles in cortical lesions of TSC and FCD IIb patients.

    However, mechanisms underlying the pathological processes of TSC and FCD IIb remain obscure. The Plexin-B2-Sema4C signalling pathway plays critical roles in neuronal morphogenesis and corticogenesis during the development of the central nervous system.

    However, the role of the Plexin-B2 system in the pathogenic process of TSC and FCD IIb has not been identified. In the present study, we investigated the expression and cell distribution characteristics of PlexinB2 and Sema4C in TSC and FCD IIb lesions with molecular technologies. Our results showed that the mRNA and protein levels of Plexin-B2 expression were significantly increased both in TSC and FCD IIb lesions versus that in the control cortex. Notably, Plexin-B2 was also predominantly observed in GCs in TSC epileptic lesions and BCs in FCD IIb lesions. In contrast, the expression of Sema4C, the ligand of Plexin-B2, was significantly decreased in DNs, GCs, and BCs in TSC and FCD IIb epileptic lesions. Additionally, Plexin-B2 and Sema4C were expressed in astrocytes and microglia cells in TSC and FCD IIb lesions. Furthermore, the expression of Plexin-B2 was positively correlated with seizure frequency in TSC and FCD IIb patients. In conclusion, our results showed the Plexin-B2-Sema4C system was abnormally expressed in cortical lesions of TSC and FCD IIb patients, signifying that the PlexinB2-Sema4C system may play a role in the pathogenic development of TSC and FCD IIb.