A novel function of proteasome

泛素-蛋白酶体途径负责选择性降解短寿命、错误折叠和有毒的蛋白质。它在调节细胞内环境方面起着重要的作用。中村西村综合征(NNS)(MIM 256040)是一种新发现的蛋白酶体功能受损的自身炎症性疾病，其致病突变和炎症症状包括周期性发热和皮疹。我们的研究结果表明，蛋白酶体活性降低与炎性细胞因子表达增加有关。泛素-蛋白酶体途径将在抗炎中发挥重要作用。这可能为其他持续性炎症性疾病的发病机制提供新的见解。

The ubiquitin-proteasome pathway is responsible for selective degradation of short-lived, misfolded and toxic proteins. It plays an important rule in tuning the intracellular environment.Nakajo-Nishimura syndrome (NNS) (MIM 256040) is an autoinflammatory disorder with impaired proteasome function, its newly identified causative mutation and inflammatory symptoms including periodic fever and skin rash.Our findings suggest that decrease of proteasome activity is associated with an increased expression of inflammatory cytokines. The ubiquitin-proteasome pathway would play an important rule against inflammation. This may provide a new insight into the pathogenesis of other persistent inflammatory diseases.