C1 inhibitor: molecular and clinical aspects

@article{Cicardi2005C1IM,
  title={C1 inhibitor: molecular and clinical aspects},
  author={Marco Cicardi and Lorenza Chiara Zingale and Andrea Zanichelli and Emanuela Pappalardo and Benedetta Cicardi},
  journal={Springer Seminars in Immunopathology},
  year={2005},
  volume={27},
  pages={286-298}
}
C1 inhibitor (C1-INH) is a serine protease inhibitor (serpins) that inactivates several different proteases in the complement, contact, coagulation, and fibrinolytic systems. By its C-terminal part (serpin domain), characterized by three β-sheets and an exposed mobile reactive loop, C1-INH binds, and blocks the activity of its target proteases. The N-terminal end (nonserpin domain) confers to C1-INH the capacity to bind lipopolysaccharides and E-selectin. Owing to this moiety, C1-INH intervenes… 
The use of plasma-derived C1 inhibitor in the treatment of hereditary angioedema
TLDR
Experienced experience with repeated weekly infusions indicates that C1-INH can be used for long-term prophylaxis in selected patients and the safety profile is excellent and there are no reports of transmission of viral infections with the preparations available at present.
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C1-INH efficiently reduced a broad spectrum of inflammatory mediators even at the lowest concentration and may thus reduce the inflammatory response in MAS.
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Two new therapies for hereditary angioedema have been developed based on the understanding that the pathogenesis of symptoms was mainly due to kallikrein activation and bradykinin release.
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A C1 inhibitor ortholog from rock bream (Oplegnathus fasciatus): molecular perspectives of a central regulator in terms of its genomic arrangement, transcriptional profiles and anti-protease activities of recombinant peptide.
TLDR
The results of this study provide the first line of evidence for the possible involvement of a teleostean C1Inh in fish immunity, based on its expressional response(s) and inhibitory properties against two enzymes involved in biological cascades.
Acquired angioedema responding to rituximab.
TLDR
This classification of AAE is of a limited value, as some patients cannot be clearly classified because they have both paraprotein and anti-C1 INH auto-antibodies, and an efficient prophylactic therapy is mandatory.
Complement aberrations and autoantibodies to complement proteins in relation to disease mechanisms.
TLDR
The complement system, a part of the innate immune system with several links to the adaptive immune system, plays an important role in the pathogenesis of many diseases and some of these mechanisms were document and clarified.
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TLDR
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TLDR
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TLDR
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TLDR
The data support the hypothesis that C1INH plays a direct role in leukocyte-endothelial cell adhesion, that the activity is mediated by carbohydrate, and that it is independent of protease inhibitory activity.
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TLDR
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TLDR
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Loop-sheet polymerization is now recognized to underlie deficiency variants of other members of the serine proteinase inhibitor (serpin) superfamily, i.e. antithrombin, C1 esterase inhibitor and alpha(1)-antichymotrypsin, which are associated with thrombosis, angio-oedema and emphysema respectively.
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TLDR
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