Role of pyrin-related molecules in vascular endothelial cells and their genetic mutations in vasculitis syndromes

RT-PCR证实炎症体成分NLRP3、ASC和原caspase-1以及吡喃在F-2小鼠血管内皮细胞中的表达。EL ISA法检测到NLRP3配体R837刺激后的F-2细胞培养上清液中IL-1β的产生，提示F-2细胞存在功能性炎症小体。内毒素可显著增加R837刺激的F-2细胞产生IL-1β。流式细胞仪分析表明，产生IL-1β的F-2细胞能与Annexin V结合，但经丝裂追踪器或溶酶追踪器标记后无明显变化。这些病例的基因分析没有发现这些分子有任何突变。这些病例的主要症状是发热、紫癜或结节性红斑，并被怀疑患有自体炎症综合征并伴有血管病变。

Expression of the inflammasome components, NLRP3, ASC and pro-caspase-1, as well as pyrin, in F-2 murine vascular endothelial cells was confirmed with RT-PCR. IL-1β production in culture media of F-2 cells after stimulation with R837, one of NLRP3 ligands, was detected by ELISA, suggesting the presence of functional inflammasome in F-2. Pretreatment with LPS significantly increased IL-1β production of R837-stimulated F-2 cells. By FACS analyses, it was revealed that F-2 cells producing IL-1β can bind Annexin V but show no change after labeling with mitotracker or lysotracker. Genetic analysis of the cases, who showed fever of unknown origin, purpura or nodular erythemas as the main symptom, and was suspected to be suffering from an autoinflammatory syndrome with vasculopathy, has not revealed any mutation in these molecules.