Prekallikrein activation and high-molecular-weight kininogen consumption in hereditary angioedema.

Abstract

Patients with hereditary angioedema lack C-1 inhibitor, a plasma alpha 2-glycoprotein that inhibits both the proteolytic action of C1, the activated first component of the complement system, and the activity of components of the contact phase of coagulation: kallikrein, factor XIa, and factor XIIa. Such patients have been shown to have low levels of C4 and C2, the natural substrates for C-1, but the levels were not correlated with the presence of symptoms. We studied three patients with angioedema for evidence of activation of the contact system and found that during a symptomatic period they had decreased levels of prekallikrein, a substrate for the activated forms of factor XII, and reductions in high-molecular-weight kininogen, a substrate for plasma kallikrein. These observations suggest that zymogens of the contact system are activated during attacks of hereditary angioedema and that some of the clinical manifestations may be mediated through products of this pathway, such as kinins.

• Authors: M Schapira, L D Silver, C F Scott, A H Schmaier, L J Prograis Jr, J G Curd, R W Colman
• Journal: The New England journal of medicine (N Engl J Med) Vol. 308 Issue 18 Pg. 1050-3 (May 05 1983) ISSN: 0028-4793 [Print] United States
• PMID: 6601240 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Kininogens
• Peptide Fragments
• Factor IX
• Factor XII
• Complement System Proteins
• Factor XI
• Prekallikrein
• Kallikreins
• Factor XIIa

Topics

• Adolescent
• Angioedema (blood, genetics, immunology)
• Blood Coagulation
• Complement System Proteins (metabolism)
• Factor IX (analysis)
• Factor XI (analysis)
• Factor XII (analysis, metabolism)
• Factor XIIa
• Female
• Humans
• Kallikreins (metabolism)
• Kininogens (metabolism)
• Male
• Middle Aged
• Molecular Weight
• Peptide Fragments (metabolism)
• Prekallikrein (metabolism)