Abstract: 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 THP-1 cells may develop good cell models for BS in vitro. The model has the advantages of simplicity, convenience, easy repetition and strong controllability, which lay a foundation for further research on the pathogenesis of BS and screening and evaluation of therapeutic drugs.

Key words: Blau syndrome, cell model, muramyl dipeptide(MDP), tumor necrosis factor-α