title: Expression of SHANK3 in the Temporal Neocortex of Patients with Intractable Temporal Epilepsy and Epilepsy Rat Models.
abstract: SH3 and multiple ankyrin (ANK) repeat domain 3 (SHANK3) is a synaptic scaffolding protein enriched in the postsynaptic density of excitatory synapses. SHANK3 plays an important role in the formation and maturation of excitatory synapses. In the brain, SHANK3 directly or indirectly interacts with various synaptic molecules including N-methyl-D-aspartate receptor, the metabotropic glutamate receptor (mGluR), and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor. Previous studies have shown that Autism spectrum disorder is a result of mutations of the main SHANK3 isoforms, which may be due to deficit in excitatory synaptic transmission and plasticity. Recently, accumulating evidence has demonstrated that overexpression of SHANK3 could induce seizures in vivo. However, little is known about the role of SHANK3 in refractory temporal lobe epilepsy (TLE). Therefore, we investigated the expression pattern of SHANK3 in patients with intractable temporal lobe epilepsy and in pilocarpine-induced models of epilepsy. Immunofluorescence, immunohistochemistry, and western blot analysis were used to locate and determine the expression of SHANK3 in the temporal neocortex of patients with epilepsy, and in the hippocampus and temporal lobe cortex of rats in a pilocarpine-induced epilepsy model. Double-labeled immunofluorescence showed that SHANK3 was mainly expressed in neurons. Western blot analysis confirmed that SHANK3 expression was increased in the neocortex of TLE patients and rats. These results indicate that SHANK3 participates in the pathology of epilepsy.
authors: Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.
authors: Yanke
authors: Y
authors: Zhang
authors: Department of Neurology, Chongqing General Hospital, Chongqing, China.
authors: Baobing
authors: B
authors: Gao
authors: Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.
authors: Yan
authors: Y
authors: Xiong
authors: Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.
authors: Fangshuo
authors: F
authors: Zheng
authors: Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.
authors: Xin
authors: X
authors: Xu
authors: Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.
authors: Yong
authors: Y
authors: Yang
authors: Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China.
authors: Yida
authors: Y
authors: Hu
authors: Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, 400016, China. xfyp@163.com.|Center of Epilepsy, Beijing Institute for Brain Disorders, Beijing, 100871, China. xfyp@163.com.|Chongqing Key Laboratory of Neurology, 1 You Yi Road, Chongqing, 400016, China. xfyp@163.com.
authors: Xuefeng
authors: X
authors: Wang
mesh_terms: D000328:Adult; D000818:Animals; D001522:Behavior, Animal; D016022:Case-Control Studies; D003710:Demography; D004195:Disease Models, Animal; D004833:Epilepsy, Temporal Lobe / Q000378:metabolism* / Q000473:pathology; D005260:Female; D006624:Hippocampus / Q000378:metabolism / Q000473:pathology; D006801:Humans; D008297:Male; D019579:Neocortex / Q000378:metabolism* / Q000473:pathology; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D017207:Rats, Sprague-Dawley; D013702:Temporal Lobe / Q000378:metabolism* / Q000473:pathology; D055815:Young Adult
chemical_list: D009419:Nerve Tissue Proteins; C492357:SHANK3 protein, human; C120532:Shank3 protein, rat
keywords: Epilepsy; SH3 and multiple ankyrin repeat domain 3; SHANK3; Temporal lobe epilepsy