实质型神经白塞病活动期 NLRP3 和 DEFA1B 的表达增强。
Enhanced NLRP3 and DEFA1B Expression During the Active Stage of Parenchymal Neuro-Behçet's Disease.
机构信息
Department of Genetics, Aziz Sancar Institute of Experimental Medicine, Istanbul University, Istanbul, Turkey.
Department of Neuroscience, Aziz Sancar Institute of Experimental Medicine, Istanbul University, Istanbul, Turkey.
出版信息
In Vivo. 2019 Sep-Oct;33(5):1493-1497. doi: 10.21873/invivo.11629.
BACKGROUND/AIM: Neurological symptoms (neuro-Behçet's disease; NBD) occur in a fraction of Behçet's disease (BD) patients and often present with parenchymal brain lesions and clinical exacerbations. Our aim was to identify genes associated with attack and remission periods of NBD.
MATERIALS AND METHODS
Microarray analysis was performed using peripheral blood mononuclear cell (PBMC) samples obtained during attack and remission periods of five NBD patients. Expression levels of the most significantly up-regulated genes were measured with real-time PCR using PBMC samples of 15 NBD patients and 20 healthy controls.
RESULTS
During NBD attacks, the most remarkably up-regulated genes were defensin alpha 1B (DEFA1B) and NLR family, pyrin domain containing 3 (NLRP3). Real time PCR studies showed significantly increased DEFA1B and NLRP3 expression levels during attacks.
CONCLUSION
Immunological factors showing the most significant increase in expression during NBD attacks were primarily associated with innate immunity functions. DEFA1B and NLRP3 can be used as biomarkers for estimation of disease activity in NBD.
背景/目的：神经系统症状（神经白塞病；NBD）发生在一小部分白塞病（BD）患者中，常表现为脑实质病变和临床恶化。我们的目的是确定与 NBD 发作和缓解期相关的基因。
材料和方法
使用 5 名 NBD 患者发作期和缓解期的外周血单核细胞（PBMC）样本进行微阵列分析。使用 15 名 NBD 患者和 20 名健康对照者的 PBMC 样本，通过实时 PCR 测量最显著上调基因的表达水平。
结果
在 NBD 发作期间，上调最明显的基因是防御素 alpha 1B（DEFA1B）和 NLR 家族，富含吡啶结构域蛋白 3（NLRP3）。实时 PCR 研究显示，在发作期间，DEFA1B 和 NLRP3 的表达水平显著增加。
结论
在 NBD 发作期间表达水平显著增加的免疫因子主要与固有免疫功能相关。DEFA1B 和 NLRP3 可作为评估 NBD 疾病活动度的生物标志物。
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