IL-1 and autoinflammatory disease: biology, pathogenesis and therapeutic targeting.

ABSTRACT: Over 20 years ago, it was first proposed that autoinflammation underpins a handful of rare monogenic disorders characterized by recurrent fever and systemic inflammation. The subsequent identification of novel, causative genes directly led to a better understanding of how the innate immune system is regulated under normal conditions, as well as its dysregulation associated with pathogenic mutations. Early on, IL-1 emerged as a central mediator for these diseases, based on data derived from patient cells, mutant mouse models and definitive clinical responses to IL-1 targeted therapy. Since that time, our understanding of the mechanisms of autoinflammation has expanded beyond IL-1 to additional innate immune processes. However, the number and complexity of IL-1-mediated autoinflammatory dise

SUBMITTER: Broderick L 

PROVIDER: S-EPMC9210802 | biostudies-literature | 2022 Aug

REPOSITORIES: biostudies-literature