Dorfleutner Andrea, Stehlik Christian
Division of Rheumatology, Department of Medicine, Feinberg School of Medicine, Robert H. Lurie Comprehensive Cancer Center, Interdepartmental Immunobiology Center and Skin Disease Research Center, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA.
Nat Immunol. 2016 Jul 19;17(8):900-2. doi: 10.1038/ni.3511.
The Pyrin inflammasome guard is disabled if the activity of small cellular GTPase is compromised, in response to defects in the mevalonate pathway and directly by mutations in Pyrin, resulting in the IL-1β-driven autoinflammatory diseases MKD and FMF.
如果小细胞GTP酶的活性受到损害,响应甲羟戊酸途径缺陷并直接由 Pyrin 突变,Pyrin炎性小体保护机制就会失效,从而导致由白细胞介素-1β驱动的自身炎症性疾病MKD和FMF。