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 ↗