Article
C1-inhibitor polymers activate the FXII-dependent kallikrein-kinin system: Implication for a role in hereditary angioedema.
Biochimica et biophysica acta - 1 Jun 2015
Madsen Daniel Elenius, Sidelmann Johannes Jakobsen, Biltoft Daniel, Gram Jørgen, Hansen Soren
Abstract excerpt
BACKGROUND: The FXII-dependent kallikrein-kinin system (KKS) is tightly regulated by the serine protease inhibitor (serpin) C1-inhibitor (C1-inh). When regulation of the FXII-dependent KKS fails, which is the case in hereditary angioedema (HAE), patients consequently experience invalidating edema attacks. HAE is caused by mutations in the C1-inh encoding gene, and we recently demonstrated that some mutations give...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.