Immunoreactive precipitation of C1 inhibitor protein from plasma of normal subjects and of patients with hereditary angioedema after isoelectric focusing.
C1-inhibitor is an acid glycoprotein, isoelectric point 3.5-3.6. Plasma of some patients with a variant form of hereditary angioedema contains high levels of functionless C1-inhibitor-albumin complex with an isoelectric point at 4.5-4.6. Therapy with Danazol, which increases C1-inhibitor levels, does not modify the isoelectric focusing pattern of such protein in patients with hereditary angioedema.
D000328:Adult; D000799:Angioedema / Q000097:blood* / Q000188:drug therapy / Q000235:genetics; D003174:Complement C1 Inactivator Proteins / Q000097:blood*; D003613:Danazol / Q000627:therapeutic use; D006801:Humans; D007122:Immunoelectrophoresis; D007525:Isoelectric Focusing; D007526:Isoelectric Point
D003174:Complement C1 Inactivator Proteins; D003613:Danazol