Molecule

PKN1/PKN2

Pyrin phosphorylating kinases

Expression change
Normal (regulatory)
Evidence level
established

Role in pathogenesis

PKN1 and PKN2 are serine-threonine kinases activated by RhoA. They directly phosphorylate pyrin at Ser208 and Ser242, promoting 14-3-3 binding and keeping pyrin inactive. FMF-associated mutant pyrin shows substantially decreased binding to PKN and 14-3-3 proteins.