The Role of Complement in Hereditary Angioedema.
Bradykinin
C1 inhibitor
Coagulation
Complement
Factor XII
Hereditary angioedema
Kallikrein
Kinin
Plasminogen
Serine protease inhibitors
Journal
Transfusion medicine reviews
ISSN: 1532-9496
Titre abrégé: Transfus Med Rev
Pays: United States
ID NLM: 8709027
Informations de publication
Date de publication:
10 2019
10 2019
Historique:
received:
13
07
2019
revised:
15
08
2019
accepted:
16
08
2019
pubmed:
5
11
2019
medline:
20
9
2020
entrez:
3
11
2019
Statut:
ppublish
Résumé
Low levels of C1 inhibitor, the main inhibitor of the classic complement system, result in paroxysmal angioedema attacks that can be incapacitating or even life-threatening in affected individuals. Molecular defects in the gene for C1 inhibitor cause hereditary angioedema. In recent years, new insights in the pathways leading to angioedema due to a deficiency of C1 inhibitor have been gathered. Bradykinin, which is formed upon activation of the kallikrein-kinin system under insufficient regulation by C1 inhibitor, plays a crucial role. Whereas C1 inhibitor also occupies a central mediatory role in other plasma systems, such as the contact activation system of coagulation and the fibrinolytic plasminogen-plasmin system, a C1 inhibitor deficiency may also cause enhanced activation of these pathways. Novel therapeutic modalities for treatment and prevention of hereditary angioedema are now available, such as different forms of C1 inhibitor concentrate and novel agents that interfere in the kallikrein-kinin system.
Identifiants
pubmed: 31676220
pii: S0887-7963(19)30104-X
doi: 10.1016/j.tmrv.2019.08.002
pii:
doi:
Substances chimiques
Complement C1 Inhibitor Protein
0
Complement Inactivating Agents
0
SERPING1 protein, human
0
Complement System Proteins
9007-36-7
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
243-247Informations de copyright
Copyright © 2019 Elsevier Inc. All rights reserved.