Article
Presence of C1-inhibitor polymers in a subset of patients suffering from hereditary angioedema.
PloS one - 1 Jan 2014
Madsen Daniel Elenius, Hansen Søren, Gram Jørgen, Bygum Anette, Drouet Christian, Sidelmann Johannes Jakobsen
Abstract excerpt
Hereditary angioedema (HAE) is a potentially life-threatening disease caused by mutations in the gene encoding the serine protease inhibitor (serpin) C1 inhibitor (C1-inh). The mutations cause decreased functional plasma levels of C1-inh, which triggers unpredictable recurrent edema attacks. Subjects suffering from HAE have been classified in type I patients with decreased functional and antigenic levels of...
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