Mutations related to HAE with normal C1INH have been described in factor XII (F12), plasminogen (PLG), angiopoietin 1 (ANGPT1), kininogen 1 (KNG1), myoferlin (MYOF), heparan sulfate (HS)-glucosamine 3-O-sulfotransferase 6 (HS3ST6), CPN1 (carboxypeptidase N1) and DAB2IP (disabled homologue 2-interacting protein) genes, with several others being studied as potentially associated with the pathogenesis of HAE [34,35,36,37]. Here, HS3ST6 is linked to hereditary angioedema.