[Inherited deficiency of the initiator molecules of the lectin-complement pathway].
The complement system is an important immune system. Its activation results in membranolytic elimination of microbes and opsonization. The classical, alternative and lectin pathways (LP) activate complement. Either mannan-binding lectin (MBL), ficolin-1, ficolin-2 or ficolin-3 initiate the LP through associated serine protease (MASP-2) after binding to microorganisms'surface carbohydrate patterns. Genetic polymorphisms behind MBL deficiency are rather common. Numerous studies indicate that MBL deficiency is a risk factor for invasive and recurrent infections, especially when other immune systems are immature, deficient or compromised. Research in ficolins is limited but last year ficolin-3 deficiency was described. This review focuses on these recently WHO defined immunodeficiencies.
Helga
H
Bjarnadottir
Bjorn Runar
BR
Ludviksson
D003167:Complement Activation / Q000235:genetics*; D037582:Complement Pathway, Mannose-Binding Lectin / Q000235:genetics*; D020022:Genetic Predisposition to Disease; D006801:Humans; D007113:Immunity, Innate / Q000235:genetics*; D007153:Immunologic Deficiency Syndromes / Q000235:genetics* / Q000276:immunology; D037102:Lectins / Q000172:deficiency / Q000235:genetics; D037241:Mannose-Binding Lectins / Q000172:deficiency / Q000235:genetics; D010641:Phenotype; D011110:Polymorphism, Genetic; D012307:Risk Factors; D000097604:Ficolins
D037102:Lectins; D037241:Mannose-Binding Lectins; D000097604:Ficolins