C1 inhibitor: just a serine protease inhibitor? New and old considerations on therapeutic applications of C1 inhibitor.
C1 inhibitor is a potent anti-inflammatory protein as it is the major inhibitor of proteases of the contact and the complement systems. C1-inhibitor administration is an effective therapy in the treatment of patients with hereditary angioedema (HAE) who are genetically deficient in C1 inhibitor. Owing to its ability to modulate the contact and complement systems and the convincing safety profile, plasma-derived C1 inhibitor is an attractive therapeutic protein to treat inflammatory diseases other than HAE. In the present review we give an overview of the biology of C1 inhibitor and its use in HAE. Furthermore, we discuss C1 inhibitor as an experimental therapy in diseases such as sepsis and myocardial infarction.
Wouters
Diana
D
Department of Immunopathology, Sanquin Research at CLB and Landsteiner Laboratory, University of Amsterdam, Academic Medical Center, Plesmanlaan 125, 1066 CX Amsterdam, The Netherlands.
Wagenaar-Bos
Ineke
I
van Ham
Marieke
M
Zeerleder
Sacha
S
D054179:Angioedemas, Hereditary / Q000188:drug therapy; D000818:Animals; D003167:Complement Activation; D003174:Complement C1 Inactivator Proteins / Q000494:pharmacology* / Q000627:therapeutic use; D050718:Complement C1 Inhibitor Protein; D001026:Coronary Artery Bypass; D006801:Humans; D009203:Myocardial Infarction / Q000188:drug therapy / Q000601:surgery; D015842:Serine Proteinase Inhibitors / Q000494:pharmacology* / Q000627:therapeutic use
D003174:Complement C1 Inactivator Proteins; D050718:Complement C1 Inhibitor Protein; C469952:SERPING1 protein, human; D015842:Serine Proteinase Inhibitors