Distinct role of IL-1β in instigating disease in Sharpin^cpdm mice.

ABSTRACT: Mice deficient in SHARPIN (Sharpin^cpdm mice), a member of linear ubiquitin chain assembly complex (LUBAC), develop severe dermatitis associated with systemic inflammation. Previous studies have demonstrated that components of the TNF-signaling pathway, NLRP3 inflammasome and IL-1R signaling are required to provoke skin inflammation in Sharpin^cpdm mice. However, whether IL-1α or IL-1β, both of which signals through IL-1R, instigates skin inflammation and systemic disease is not known. Here, we have performed extensive cellular analysis of pre-diseased and diseased Sharpin^cpdm mice and demonstrated that cellular dysregulation precedes skin inflammation. Furthermore, we demonstrate a specific role for IL-1β, but not IL-1α, in instigating dermatitis in Sharpin<

SUBMITTER: Gurung P 

PROVIDER: S-EPMC5125001 | biostudies-literature | 2016 Nov

REPOSITORIES: biostudies-literature