Establishment of cell models for Blau syndrome

• Published in: Ji chu yi xue yu lin chuang = Jichu yixue yu linchuang = Basic medical sciences and clinics Vol. 42; no. 3; pp. 406 - 410
• Main Author: SONG Hao-xin, YE Cai-ying, ZHU Lei
• Format: Journal Article
• Language: Chinese
• Published: Institute of Basic Medical Sciences and Peking Union Medical College Hospital, Chinese Academy of Medical Sciences / Peking Union Medical College 01-03-2022
• Subjects: blau syndrome|cell model|muramyl dipeptide(mdp)|tumor necrosis factor-α
• ISSN: 1001-6325
• DOI: 10.16352/j.issn.1001-6325.2022.03.036

Objective To establish a stable and reliable cell models of Blau syndrome (BS)in vitro. Methods RAW264.7, iBMDM and THP-1 cells were used as the research objects and then stimulated by muramyl dipeptide (MDP) or L18-MDP. Meanwhile, positive drugs etanercept (ETN) and GSK583 treatment groups were set to investigate the response of the model to evaluate effectiveness. Cell culture supernatant was collected after 22 h, and the level of tumor necrosis factor-α (TNF-α) was determined by enzyme linked immunosorbent assay (ELISA). Results The secretion of TNF-α from RAW264.7 and iBMDM cells was significantly increased after stimulation by 10 μg/mL MDP(P＜0.01), while ETN and GSK583 inhibited the increase of TNF-α induced by MDP(P＜0.01). After THP-1 cells were induced into THP-1-Mφ by PMA, 0.2 and 1 μg/mL L18-MDP increased the level of TNF-α in the culture supernatant(P＜0.01). When ETN was added along with 0.2 μg/mL L18-MDP, the secretion of TNF-α was significantly inhibited (P＜0.01). Conclusions RAW264.7,iBMDM and TH