title: Expression and cellular distribution of transient receptor potential vanilloid 4 in cortical tubers of the tuberous sclerosis complex.
abstract: Cortical tubers in patients with tuberous sclerosis complex (TSC) are highly associated with intractable epilepsy. Recent evidence has shown that transient receptor potential vanilloid 4 (TRPV4) has direct effects on both neurons and glial cells. To understand the role of TRPV4 in pathogenesis of cortical tubers, we investigated the expression patterns of TRPV4 in cortical tubers of TSC compared with normal control cortex (CTX). We found that TRPV4 was clearly up-regulated in cortical tubers at the protein levels. Immunostaining indicated that TRPV4 was specially distributed in abnormal cells, including dysplastic neurons (DNs) and giant cells (GCs). In addition, double immunofluorescent staining revealed that TRPV4 was localized on neurofilament proteins (NF200) positive neurons and glial fibrillary acidic portein (GFAP) positive reactive astrocytes. Moreover, TRPV4 co-localized with both glutamatergic and GABAergic neurons. Furthermore, protein levels of protein kinase C (PKC), but not protein kinase A (PKA), the important upstream factors of the TRPV4, were significantly increased in cortical tubers. Taken together, the overexpression and distribution patterns of TRPV4 may be linked with the intractable epilepsy caused by TSC.
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Xin
authors.initials: X
authors.lastname: Chen
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Meihua
authors.initials: M
authors.lastname: Yang
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Feiji
authors.initials: F
authors.lastname: Sun
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Chao
authors.initials: C
authors.lastname: Liang
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Yujia
authors.initials: Y
authors.lastname: Wei
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Lukang
authors.initials: L
authors.lastname: Wang
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Jiong
authors.initials: J
authors.lastname: Yue
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Bing
authors.initials: B
authors.lastname: Chen
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China.
authors.forename: Song
authors.initials: S
authors.lastname: Li
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China. Electronic address: liushi2425@163.com.
authors.forename: Shiyong
authors.initials: S
authors.lastname: Liu
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China. Electronic address: huiyang64@sohu.com.
authors.forename: Hui
authors.initials: H
authors.lastname: Yang
mesh_terms: D002540:Cerebral Cortex / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D017868:Cyclic AMP-Dependent Protein Kinases / Q000378:metabolism; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D018698:Glutamic Acid / Q000378:metabolism; D006801:Humans; D007223:Infant; D008297:Male; D016900:Neurofilament Proteins / Q000378:metabolism; D011493:Protein Kinase C / Q000378:metabolism; D012640:Seizures / Q000209:etiology; D050053:TRPM Cation Channels / Q000378:metabolism*; D014402:Tuberous Sclerosis / Q000150:complications / Q000473:pathology*; D005680:gamma-Aminobutyric Acid / Q000378:metabolism
chemical_list: D005904:Glial Fibrillary Acidic Protein; D016900:Neurofilament Proteins; D050053:TRPM Cation Channels; C437888:TRPM4 protein, human; C051774:neurofilament protein H; D018698:Glutamic Acid; D005680:gamma-Aminobutyric Acid; D017868:Cyclic AMP-Dependent Protein Kinases; D011493:Protein Kinase C
keywords: Epilepsy; Protein kinase A; Protein kinase C; Transient receptor potential vanilloid 4; Tuberous sclerosis complex