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Toll-like receptors pathway in common variable immune deficiency (CVID) and X-linked agammaglobulinemia (XLA).
European Cytokine Network ( IF 2.2 ) Pub Date : 2019-04-30 , DOI: 10.1684/ecn.2018.0420
Parsova Tavasolian 1 , Laleh Sharifi 2 , Asghar Aghamohammadi 3 , Farshid Noorbakhsh 1 , Rouzbeh Sanaei 4 , Mahsima Shabani 5 , Nima Rezaei 6
Affiliation  

Common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA) are two major humoral immunodeficiencies, causing a high rate of early age mortality in children. In order to identifiy the possible factors involved in the pathogenesis of CVID and XLA, recent studies have focused on Toll-like receptors (TLRs) and demonstrate the defects in different TLR pathways in immune cells of CVID and XLA patients. Herein, we measured TLR-4 and TLR-9 RNA levels and consequently TNF-α and IFN-α production in peripheral blood mononuclear cells (PBMCs) of patients with CVID and XLA. Contrary to healthy individuals, TLR-9 expression was not significantly increased after ligand stimulation, whereas ligand-induced TLR-4 expression was not significantly different from that in healthy control PBMCs. Lipopolysaccharide (LPS)-stimulated TNF-α production was significantly reduced in patients compared to controls, whereas IFN-α production was increased in all groups after CpG stimulation without any significant inter-group difference. Our data suggest that defects in TLR-9 activated pathways may be a result of the decreased TLR-9 expression, although TLR-9 is not the only modulator of IFN-α production in these patients. On the other hand, impaired signaling in TLR-4 activated pathways which results in significant reduction in TNF-α production are not related to a defect in TLR-4 expression.

中文翻译:

常见的可变免疫缺陷症(CVID)和X连锁的丙种球蛋白血症(XLA)中的Toll样受体途径。

常见的可变免疫缺陷(CVID)和X连锁的丙种球蛋白血症(XLA)是两种主要的体液免疫缺陷,导致儿童的早期死亡率很高。为了确定可能与CVID和XLA发病机理有关的因素,最近的研究集中在Toll样受体(TLR),并证明了CVID和XLA患者免疫细胞中不同TLR途径的缺陷。在本文中,我们测量了CVID和XLA患者的外周血单核细胞(PBMC)中TLR-4和TLR-9 RNA的水平,以及由此产生的TNF-α和IFN-α。与健康个体相反,配体刺激后TLR-9表达没有显着增加,而配体诱导的TLR-4表达与健康对照PBMC中的表达没有显着差异。与对照组相比,患者的脂多糖(LPS)刺激的TNF-α产量显着降低,而CpG刺激后所有组的IFN-α产量均增加,而组间无显着差异。我们的数据表明,尽管TLR-9并不是这些患者中唯一的IFN-α产生调节剂,但TLR-9激活途径的缺陷可能是TLR-9表达降低的结果。另一方面,导致TNF-α产生显着减少的TLR-4激活途径中的信号传导受损与TLR-4表达缺陷无关。我们的数据表明,尽管TLR-9并不是这些患者中唯一的IFN-α产生调节剂,但TLR-9激活途径的缺陷可能是TLR-9表达降低的结果。另一方面,导致TNF-α产生显着减少的TLR-4激活途径中的信号传导受损与TLR-4表达缺陷无关。我们的数据表明,尽管TLR-9不是这些患者中唯一的IFN-α产生调节剂,但TLR-9激活途径的缺陷可能是TLR-9表达降低的结果。另一方面,导致TNF-α产生显着减少的TLR-4激活途径中的信号传导受损与TLR-4表达缺陷无关。
更新日期:2019-04-30
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