Article
Mechanisms of C1-inhibitor deficiency.
Immunobiology - 1 Sept 2002
Pappalardo Emanuela, Zingale Lorenza C, Terlizzi Adelaide, Zanichelli Andrea, Folcioni Anna, Cicardi Marco
Abstract excerpt
C1 inhibitor (C1-Inh) is a protease inhibitor of the serpin family. It interacts and forms complexes with several serine proteases although not all these interactions were proved to be relevant in vivo. Based on studies in deficient patients, C1-Inh appears pivotal in regulating the activation of complement classical pathway and of contact system. The best recognized consequence of defective C1-Inh function is...
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