Expression of pannexin 1 and 2 in cortical lesions from intractable epilepsy patients with focal cortical dysplasia
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Song Li1, Zhenle Zang1, Jiaojiang He2, Xin Chen1, Sixun Yu3, Yuchun Pei1, Zhi Hou1, Ning An1, Hui Yang1, Chunqing Zhang1, Shiyong Liu1
1Epilepsy Research Center of PLA, Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing 400037, China
2Department of Neurosurgery, General Hospital of the People's Liberation Army Lanzhou Military Region, Lanzhou, Gansu, 730050, China
3Department of Neurosurgery, General Hospital of the People's Liberation Army Chengdu Military Region, Chengdu, Sichuan, 610083, China
Chunqing Zhang, email: email@example.com
Shiyong Liu, email: firstname.lastname@example.org
Keywords: focal cortical dysplasia, epileptogenesis, pathogenesis, pannexin 1, pannexin 2
Received: May 16, 2016 Accepted: December 16, 2016 Published: December 28, 2016
Focal cortical dysplasia (FCD) is a major cause of intractable epilepsy in children however the mechanisms underlying the pathogenesis of FCD and FCD induced epilepsy remain unclear. Increasing evidence suggests that the large-pore ion channels, pannexin 1 (Panx1) and 2 (Panx2), are involved in epilepsy and brain development. In this study, we investigated the expression of Panx1 and Panx2 in surgical samples from patients with FCD type Ia (FCDIa), type IIa (FCDIIa), and type IIb (FCDIIb) and in age-matched autopsy control samples. We found Panx1 mRNA and protein levels were both increased in all these FCD samples. Immunohistochemical analyses revealed that Panx1 was mainly distributed in microcolumn neurons, dysmorphic neurons (DNs), balloon cells (BCs) and reactive astrocytes. Double-labeled staining showed that the Panx1-positive neurons were mostly glutamatergic DNs and occasionally GABAergic normal-appearing neurons. Importantly, the protein levels of Panx1 positively correlated with the frequency of seizures. Intriguingly, the Panx2 mRNA and protein levels were only upregulated in FCDIIb lesions and characteristically expressed on SOX2-positive multipotential BCs. Immunofluorescent experiments identified that Panx2-positive BCs mainly expressed the neuronal differentiation transcription factor MASH1 but not the immature glial marker vimentin. Taken together, our results established a potential role of the specific expression and cellular distribution patterns of Panx1 and Panx2 in FCD-associated epileptogenesis and pathogenesis.
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