Fig. 7From: Molecular biology of autoinflammatory diseasesActivation of pyrin inflammasome in patients with hyper-IgD syndrome (HIDS). Deficiency of MVK activity by loss-of-function mutations leads to depletion of geranylgeranyl pyrophosphate, resulting in the inactivation of RhoA and following activation of PKN1/2. This causes activation of pyrin inflammasomeBack to article page