Molecule

RhoA GTPase

Upstream guard target

Expression change
Normal (sensor target)
Evidence level
established

Role in pathogenesis

Pyrin functions as a guard for RhoA GTPase activity. When RhoA is inactivated by bacterial toxins, pyrin detects this as a danger signal. RhoA normally activates PKN1/PKN2 kinases that keep pyrin phosphorylated and inactive. The RhoA-PKN-pyrin axis is the central regulatory pathway disrupted in FMF.

Sources (3)

DetailsPark YH et al. (2016) Pyrin inflammasome activation and RhoA signaling in the autoinflammatory diseases FMF and HIDSDOI
DetailsSchnappauf O et al. (2019) The pyrin inflammasome in health and diseaseDOI
DetailsHeilig R et al. (2018) Function and mechanism of the pyrin inflammasomeDOI