RhoA GTPase

Upstream guard target

Expression
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.