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

用RT-PCR证实F-2小鼠血管内皮细胞中炎性体组分NLRP 3、ASC和前半胱天冬酶-1以及pyrin的表达。ELISA法检测NLRP 3配体R837刺激F-2细胞后培养液中IL-1β的产生，提示F-2细胞中存在功能性炎性小体。LPS预处理显着增加R837刺激的F-2细胞IL-1β的产生。流式细胞仪分析显示，产生IL-1β的F-2细胞可与Annexin V结合，但用mitotracker或lysotracker标记后，这种结合没有变化。对这些病例的基因分析显示，这些病例的主要症状是发热原因不明、紫癜或结节性紫癜，并被怀疑患有伴有血管病变的自身炎症综合征，但没有发现这些分子的任何突变。

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.