title: Down-Regulated Expression of Liver X Receptor beta in Cortical Lesions of Patients with Focal Cortical Dysplasia.
abstract: Focal cortical dysplasia (FCD) is strongly associated with medically intractable epilepsy. Studies suggest that liver X receptor beta (LXRβ) may participate in the pathogenesis of FCD. The present study investigated the expression pattern of LXRβ in FCD and the distribution of LXRβ in different neural precursor cells. Twenty-five surgical specimens from FCD patients and 11 age-matched control samples from autopsies were included in our study. Protein levels and distribution were detected by western blot, immunohistochemistry, and immunofluorescence staining. We found that (1) the level of LXRβ protein was markedly reduced in FCD. (2) LXRβ staining was weaker in the dysplastic cortices of FCD and was mainly observed in neuronal microcolumns, and malformed cells. (3) LXRβ was co-localized with radial glial cells (RGCs) markers and oligodendrocyte precursor cells (OPCs) markers in malformed cells. (4) RGCs marker and OPCs marker were down-regulated while LXRβ downstream factors were up-regulated in FCD specimens. Taken together, our results indicate that LXRβ may interact with β-catenin to regulate the generation of OPCs and the transformation of RGCs. LXRβ therefore potentially contributes to the pathogenesis of FCD.
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, 183 Xinqiao Main Street, Shapingba District, Chongqing, 400037, China.
authors.forename: Xin
authors.initials: X
authors.lastname: Chen
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, 183 Xinqiao Main Street, Shapingba District, Chongqing, 400037, China.
authors.forename: Lukang
authors.initials: L
authors.lastname: Wang
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, 183 Xinqiao Main Street, Shapingba District, Chongqing, 400037, China.
authors.forename: Bing
authors.initials: B
authors.lastname: Chen
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, 183 Xinqiao Main Street, Shapingba District, Chongqing, 400037, China.
authors.forename: Jiong
authors.initials: J
authors.lastname: Yue
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, 183 Xinqiao Main Street, Shapingba District, Chongqing, 400037, China.
authors.forename: Gang
authors.initials: G
authors.lastname: Zhu
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, 183 Xinqiao Main Street, Shapingba District, Chongqing, 400037, China.
authors.forename: Chunqing
authors.initials: C
authors.lastname: Zhang
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, 183 Xinqiao Main Street, Shapingba District, Chongqing, 400037, China.
authors.forename: Shiyong
authors.initials: S
authors.lastname: Liu
authors.affiliation: Department of Neurosurgery, Xinqiao Hospital, Third Military Medical University, 183 Xinqiao Main Street, Shapingba District, Chongqing, 400037, China. huiyang64@sohu.com.
authors.forename: Hui
authors.initials: H
authors.lastname: Yang
mesh_terms: D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D015536:Down-Regulation; D005260:Female; D006801:Humans; D000071518:Liver X Receptors / Q000235:genetics / Q000378:metabolism*; D008297:Male; D054220:Malformations of Cortical Development / Q000378:metabolism*; D058953:Neural Stem Cells / Q000378:metabolism; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D009836:Oligodendroglia / Q000378:metabolism
chemical_list: D000071518:Liver X Receptors
keywords: Focal cortical dysplasia; LXRβ; Oligodendrocyte precursor cell; Radial glial cell