Four strains of TRAPS mutant model mice (T79M, G87V, C62Y, and T90I mutants) were used for analysis. When LPS+D-Galactosamine was administered as inflammatory stimuli, lethal responses were observed in wild-type and T90I mice, while the lethal responses were reduced in T79M and G87V mutant mice. In experiments using mouse bone marrow-derived macrophages, T79M and G87V mutant cells showed decreased responsiveness to TNF, and the cell surface expression of TNFR1 was reduced. The stimuli examined in this study did not reproduce the inflammatory pathology observed in TRAPS patients, suggesting the presence of other pathology-specific proinflammatory factors.
