Kinin formation in hereditary angioedema plasma: evidence against kinin derivation from C2 and in support of “spontaneous” formation of bradykinin

T Fields, B Ghebrehiwet, AP Kaplan - Journal of Allergy and Clinical …, 1983 - Elsevier
Hereditary angioedema (HAE) is due to a functional deficiency of the inhibitor of the
activated first component of complement (C 1 INH). This abnormality is thought to be
responsible for the generation of a kininlike peptide in HAE plasma that is derived from the
second component of complement (C2). Specifically, a combination of C2 cleavage by C 1s
and C2 fragment cleavage by plasmin has been reported to generate a kinin that is
distinguishable from bradykinin. We have attempted to generate this peptide by activating …