Mast cells mediate neutrophil recruitment and vascular leakage through the NLRP3 inflammasome in histamine-independent urticaria.

ABSTRACT: Urticarial rash observed in cryopyrin-associated periodic syndrome (CAPS) caused by nucleotide-binding oligomerization domain-leucine-rich repeats containing pyrin domain 3 (NLRP3) mutations is effectively suppressed by anti-interleukin (IL)-1 treatment, suggesting a pathophysiological role of IL-1beta in the skin. However, the cellular mechanisms regulating IL-1beta production in the skin of CAPS patients remain unclear. We identified mast cells (MCs) as the main cell population responsible for IL-1beta production in the skin of CAPS patients. Unlike normal MCs that required stimulation with proinflammatory stimuli for IL-1beta production, resident MCs from CAPS patients constitutively produced IL-1beta. Primary MCs expressed inflammasome components and secreted IL-1beta via NLRP3 and apo

SUBMITTER: Nakamura Y 

PROVIDER: S-EPMC2715029 | biostudies-literature | 2009 May

REPOSITORIES: biostudies-literature