In patients with HAE with nC1-INH 6 pathogenic variants have been identified in factor XII (HAE-FXII), angiopoietin-1 (HAE-ANGPT1), plasminogen (HAE-PLG), kininogen 1 (HAE-KNG1), myoferlin (HAE-MYOF), and heparan sulfate-glucosamine 3-O-sulfotransferase 6 (HAE-HS3ST6) [2]. Here, KNG1 is linked to hereditary angioedema.