title: Contribution of the inflammasomes to autoinflammatory diseases and recent mouse models as research tools.
abstract: Inflammasomes are multiprotein complexes that serve as activating platforms for the enzyme caspase-1 in response to various danger signals. Active caspase-1 can cleave the precursors of the pro-inflammatory cytokines IL-1β and IL-18 and thereby activate them. Deregulation of this cascade caused by mutations in genes coding for inflammasomal components and their interaction partners can lead to severe disease. This review summarizes the contribution of deregulated inflammasomes to the field of autoinflammatory syndromes. In addition, it gives insight into currently available mouse models that are used to study and characterize the role of the inflammasome components in the pathophysiology of these diseases.
authors: Department of Pediatrics, University Hospital Carl Gustav Carus, Fetscherstrasse 74, 01307 Dresden, Germany. michael.heymann@uniklinikum-dresden.de
authors: Michael C
authors: MC
authors: Heymann
authors: Angela
authors: A
authors: Rösen-Wolff
mesh_terms: D000152:Acne Vulgaris / Q000235:genetics / Q000276:immunology; D000818:Animals; D001170:Arthritis, Infectious / Q000235:genetics / Q000276:immunology; D002352:Carrier Proteins / Q000235:genetics; D020170:Caspase 1 / Q000235:genetics / Q000378:metabolism; D056587:Cryopyrin-Associated Periodic Syndromes / Q000235:genetics / Q000276:immunology; D003598:Cytoskeletal Proteins / Q000235:genetics; D004195:Disease Models, Animal; D056660:Hereditary Autoinflammatory Diseases / Q000276:immunology*; D006801:Humans; D058847:Inflammasomes / Q000235:genetics* / Q000276:immunology*; D007249:Inflammation / Q000235:genetics; D020382:Interleukin-18 / Q000378:metabolism; D053583:Interleukin-1beta / Q000378:metabolism; D051379:Mice; D008822:Mice, Transgenic; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; D017511:Pyoderma Gangrenosum / Q000235:genetics / Q000276:immunology; D000071198:Pyrin
chemical_list: D002352:Carrier Proteins; D003598:Cytoskeletal Proteins; D058847:Inflammasomes; D020382:Interleukin-18; D053583:Interleukin-1beta; D000071199:NLR Family, Pyrin Domain-Containing 3 Protein; C492716:Nlrp3 protein, mouse; D000071198:Pyrin; D020170:Caspase 1