Activated mast cells trigger edema in allergic and inflammatory disease. We report a paracrine mechanism by which mast cell-released heparin increases vascular permeability in vivo. Heparin activated the protease factor XII, which initiates bradykinin formation in plasma. Targeting factor XII or kinin B2 receptors abolished heparin-triggered leukocyte-endothelium adhesion and interfered with a ...

The protocol consists of running a native gel with in-gel digestion by proteases, subsequent mass spectrometrical determination of protein sequence and modifications, followed by electro-elution and conformational analysis using melting point and circular dichroism. Finally, the eluted protein is tested for preserved function. Herein, C1 esterase inhibitor is applied on a native gel; ingel dige...

Acute angioedema attacks are conventionally treated with antihistamines and steroids, in line with a presumed mechanism of disease involving overwhelming mast-cell degranulation. This approach overlooks a small but important minority of cases in which attacks are bradykinin driven and exhibit poor responsiveness to steroid or anti-histamine therapy. These patients may have a family history of a...

Hereditary angioedema (HAE), a rare autosomal dominant disorder, was first described in the late 19th century. The disease remained poorly understood and without therapeutic options until the latter half of the 20th century. Advances in the understanding of immunologic and hematologic pathways have shed light on HAE, a disease characterized by painful and unpredictable recurrent attacks of nonp...

Department of Medicine, Division of Clinical Immunology and Allergy, McMaster University, Hamilton, Ontario, Canada, L8N 3Z5 We report a 64 year-old woman with longstanding hereditary angioedema who previously required a tracheotomy. In September 2005, while receiving prophylactic Danazol she noticed a left sided swelling involving her throat and uvula that was of moderate intensity by 1110h. S...

C1 inhibitor of serine proteases (C1-INH) performs a regulatory function in the complement system and vascular permeability. Deficiency C1-INH leads to various forms angioedema, including hereditary angioedema (HAE). The cause HAE is genetically determined violation synthesis C1-INH. A decrease level 50% relative norm an increase production bradykinin, which basis for diagnosis HAE. development...

Background Hereditary angioedema (HAE) types I and II (due to quantitative and qualitative C1-INH deficiency, respectively) is a rare autosomal dominant condition in which more than 300 different mutations in the entire C1-INH gene (SERPING1) have been described. The objective of this study is to identify and characterize the mutation in the SERPING1 gene in a family of HAE outpatients from the...