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.
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Acknowledgments
The investigators would like to thank the technicians Jin Peng, Qian Chen, and Wen-Qiang Cai (Central Laboratory of Xinqiao Hospital, Third Military Medical University, Chongqing, People’s Republic of China), for their excellent assistance with the laser scanning confocal microscopy. This study was supported by National Natural Science Foundation of China (No. 81271436 and No. 81370028).
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Chen, X., Wang, L., Chen, B. et al. Down-Regulated Expression of Liver X Receptor beta in Cortical Lesions of Patients with Focal Cortical Dysplasia. J Mol Neurosci 60, 223–231 (2016). https://doi.org/10.1007/s12031-016-0795-7
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DOI: https://doi.org/10.1007/s12031-016-0795-7