Genomes and Genes
Gene Symbol: Masp2
Description: mannan-binding lectin serine peptidase 2
Alias: MASP-2, MAp19, mannan-binding lectin serine protease 2, MBL-associated serine protease 2, mannose binding lectin-associated serine protease-2, mannose-binding protein-associated serine protease 2
- Stover C, Thiel S, Lynch N, Schwaeble W. The rat and mouse homologues of MASP-2 and MAp19, components of the lectin activation pathway of complement. J Immunol. 1999;163:6848-59 pubmed..activation, a serine protease of 76 kDa, termed MASP-2, and a MASP-2 related plasma protein of 19 kDa, termed MAp19. Upon activation of the MBL/MASPs/MAp19 complex, MASP-2 cleaves the fourth complement component C4, while the role ..
- Endo Y, Nakazawa N, Liu Y, Iwaki D, Takahashi M, Fujita T, et al. Carbohydrate-binding specificities of mouse ficolin A, a splicing variant of ficolin A and ficolin B and their complex formation with MASP-2 and sMAP. Immunogenetics. 2005;57:837-44 pubmed..These results suggest that ficolin A and its variant function as recognition molecules of the lectin pathway, and ficolin B plays a distinct role through its unique carbohydrate-binding specificity. ..
- Wallis R, Lynch N, Roscher S, Reid K, Schwaeble W. Decoupling of carbohydrate binding and MASP-2 autoactivation in variant mannose-binding lectins associated with immunodeficiency. J Immunol. 2005;175:6846-51 pubmed..Analogous molecular defects in human MBL probably combine to create the mutant phenotypes of immunodeficient individuals. ..
- Venkatraman Girija U, Furze C, Toth J, Schwaeble W, Mitchell D, Keeble A, et al. Engineering novel complement activity into a pulmonary surfactant protein. J Biol Chem. 2010;285:10546-52 pubmed publisher..Thus, the active rather than the zymogen state is default in lectin.MASP complexes and must be inhibited through additional regions in circulating MBLs until triggered by pathogen recognition. ..
- Kidmose R, Laursen N, Dobó J, Kjaer T, Sirotkina S, Yatime L, et al. Structural basis for activation of the complement system by component C4 cleavage. Proc Natl Acad Sci U S A. 2012;109:15425-30 pubmed..Our results provide a unified structural framework for understanding the early and essential step of C4 cleavage in the elimination of pathogens and altered self through two major pathways of complement activation. ..
- Møller Kristensen M, Thiel S, Sjoholm A, Matsushita M, Jensenius J. Cooperation between MASP-1 and MASP-2 in the generation of C3 convertase through the MBL pathway. Int Immunol. 2007;19:141-9 pubmed
- Thiel S, Vorup Jensen T, Stover C, Schwaeble W, Laursen S, Poulsen K, et al. A second serine protease associated with mannan-binding lectin that activates complement. Nature. 1997;386:506-10 pubmed..Thus complement activation through MBL, like the classical pathway, involves two serine proteases and may antedate the development of the specific immune system of vertebrates. ..
- Ambrus G, Gal P, Kojima M, Szilágyi K, Balczer J, Antal J, et al. Natural substrates and inhibitors of mannan-binding lectin-associated serine protease-1 and -2: a study on recombinant catalytic fragments. J Immunol. 2003;170:1374-82 pubmed..Nevertheless, relative rates of reaction with alpha-2-macroglobulin and C1-inhibitor suggest that alpha-2-macroglobulin may be a significant physiological inhibitor of MASP-1. ..
- Wallis R, Dodds A, Mitchell D, Sim R, Reid K, Schwaeble W. Molecular interactions between MASP-2, C4, and C2 and their activation fragments leading to complement activation via the lectin pathway. J Biol Chem. 2007;282:7844-51 pubmed