Polymorphisms in Mannan-Binding Lectin (MBL)-associated serine protease 2 affect stability, binding to MBL, and enzymatic activity

Journal of Immunology

Volumn 182, Issue 5, 2009, Pages 2939-2947

  Thiel, Steffen ^a   Kolev, Martin ^a   Degn, Søren ^a   Steffensen, Rudi ^b   Hansen, Annette G ^a   Ruseva, Marieta ^a   Jensenius, Jens C ^a  

^a AARHUS UNIVERSITY   (Denmark)

^b AALBORG UNIVERSITY HOSPITAL   (Denmark)

Author keywords

[No Author keywords available]

Indexed keywords

COMPLEMENT COMPONENT C4 BINDING PROTEIN; MANNAN; MANNAN BINDING LECTIN; MANNAN BINDING LECTIN ASSOCIATED SERINE PROTEINASE; MANNAN BINDING LECTIN ASSOCIATED SERINE PROTEINASE 2; UNCLASSIFIED DRUG; COMPLEMENT COMPONENT C4; MANNOSE BINDING LECTIN; MASP2 PROTEIN, HUMAN; RECOMBINANT PROTEIN;

AFRICAN AMERICAN; ALLOTYPE; ARTICLE; CONTROLLED STUDY; DNA POLYMORPHISM; ENZYME ACTIVITY; GEL PERMEATION CHROMATOGRAPHY; GENE FREQUENCY; GENE MUTATION; HETEROZYGOTE; HUMAN; HUMAN CELL; PRIORITY JOURNAL; PROTEIN BINDING; PROTEIN FOLDING; PROTEIN LOCALIZATION; AMINO ACID SUBSTITUTION; BIOSYNTHESIS; CELL LINE; COMPLEMENT LECTIN PATHWAY; DRUG ANTAGONISM; ENZYME ACTIVATION; ENZYME STABILITY; GENETIC PREDISPOSITION; GENETIC VARIABILITY; GENETICS; GENOTYPE; IMMUNOLOGY; METABOLISM; SECRETION; SINGLE NUCLEOTIDE POLYMORPHISM;

AMINO ACID SUBSTITUTION; CELL LINE; COMPLEMENT C4; COMPLEMENT PATHWAY, MANNOSE-BINDING LECTIN; ENZYME ACTIVATION; ENZYME STABILITY; GENETIC PREDISPOSITION TO DISEASE; GENETIC VARIATION; GENOTYPE; HUMANS; MANNOSE-BINDING LECTIN; MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES; POLYMORPHISM, SINGLE NUCLEOTIDE; PROTEIN BINDING; RECOMBINANT PROTEINS;

EID: 64849114540     PISSN: 00221767     EISSN: 15506606     Source Type: Journal    
DOI: 10.4049/jimmunol.0802053     Document Type: Article

References (31)

1. Walport, M. J. 2001. Complement. N. Engl. J. Med. 344: 1058-1066.
2. Markiewski, M. M., and J. D. Lambris. 2007. The role of complement in inflammatory diseases from behind the scenes into the spotlight. Am. J. Pathol. 171: 715-727.
3. Thiel, S. 2007. Complement activating soluble pattern recognition molecules with collagen-like regions, mannan-binding lectin, ficolins and associated proteins. Mol. Immunol. 44: 3875-3888.
4. Fujita, T. 2002. Evolution of the lectin-complement pathway and its role in innate immunity. Nat. Rev. Immunol. 2: 346-353.
5. Wallis, R. 2007. Interactions between mannose-binding lectin and MASPs during complement activation by the lectin pathway. Immunobiology 212: 289-299.
6. Stover, C. M., S. Thiel, M. Thelen, N. J. Lynch, T. Vorup-Jensen, J. C. Jensenius, and W. J. Schwaeble. 1999. Two constituents of the initiation complex of the mannan-binding lectin activation pathway of complement are encoded by a single structural gene. J. Immunol. 162: 3481-3490.
7. Matsushita, M., and T. Fujita. 1992. Activation of the classical complement pathway by mannose-binding protein in association with a novel C1s-like serine protease. J. Exp. Med. 176: 1497-1502.
8. Ambrus, G., P. Gal, M. Kojima, K. Szilagyi, J. Balczer, J. Antal, L. Graf, A. Laich, B. E. Moffatt, W. Schwaeble, et al. 2003. Natural substrates and inhibitors of mannan-binding lectin-associated serine protease-1 and -2: a study on recombinant catalytic fragments. J. Immunol. 170: 1374-1382.
9. Thiel, S., T. Vorup-Jensen, C. M. Stover, W. Schwaeble, S. B. Laursen, K. Poulsen, A. C. Willis, P. Eggleton, S. Hansen, U. Holmskov, et al. 1997. A second serine protease associated with mannan-binding lectin that activates complement. Nature 386: 506-510.
10. Matsushita, M., S. Thiel, J. C. Jensenius, I. Terai, and T. Fujita. 2000. Proteolytic activities of two types of mannose-binding lectin-associated serine protease. J. Immunol. 165: 2637-2642.
11. Thiel, S., R. Steffensen, I. J. Christensen, W. K. Ip, Y. L. Lau, I. J. Reason, H. Eiberg, M. Gadjeva, M. Ruseva, and J. C. Jensenius. 2007. Deficiency of mannan-binding lectin associated serine protease-2 due to missense polymorphisms. Genes Immun. 8: 154-163.
12. Stengaard-Petersen, K., S. Thiel, M. Gadjeva, M. Moller-Kristensen, R. Sorensen, L. T. Jensen, A. G. Sjöholm, L. Fugger, and J. C. Jensenius. 2003. Inherited deficiency of mannan-binding lectin-associated serine protease 2. N. Engl. J. Med. 349: 554-560.
13. Møller-Kristensen, M., J. C. Jensenius, L. Jensen, N. Thielens, V. Rossi, G. Arlaud, and S. Thiel. 2003. Levels of mannan-binding lectin-associated serine protease-2 in healthy individuals. J. Immunol. Methods 282: 159-167.
14. Thiel, S., S. V. Petersen, T. Vorup-Jensen, M. Matsushita, T. Fujita, C. M. Stover, W. Schwaeble, and J. C. Jensenius. 2000. Interaction of C1q and mannan-binding lectin (MBL) with C1r.C1s, MBL-associated serine proteases 1 and 2, and the MBL-associated protein MAp19. J. Immunol. 165: 878-887.
15. Petersen, S. V., S. Thiel, L. Jensen, R. Steffensen, and J. C. Jensenius. 2001. An assay for the mannan-binding lectin pathway of complement activation. J. Immunol. Methods 257: 107-116.
16. Thiel, S., M. Møller-Kristensen, L. Jensen, and J. C. Jensenius. 2002. Assays for the functional activity of the mannan-binding lectin pathway of complement activation. Immunobiology 205: 446-454.
17. Chen, C. B., and J. Wallis. 2001. Stoichiometry of complexes between mannosebinding protein and its associated serine proteases: defining functional units for complement activation. J. Biol. Chem. 276: 25894-25902.
18. Gregory, L. A., N. M. Thielens, M. Matsushita, R. Sorensen, G. J. Arlaud, J. C. Fontecilla-Camps, and C. Gaboriaud. 2004. The x-ray structure of human mannan-binding lectin-associated protein 19 (MAp19) and its interaction site with mannan-binding lectin and L-ficolin. J. Biol. Chem. 279: 29391-29397.
19. Møller-Kristensen, M., S. Thiel, A. G. Hansen, and J. C. Jensenius. 2003. On the site of C4 deposition upon complement activation via the mannan-binding lectin pathway or the classical pathway. Scand. J. Immunol. 57: 556-561.
20. Vorup-Jensen, T., S. V. Petersen, A. G. Hansen, K. Poulsen, W. Schwaeble, R. B. Sim, K. B. M. Reid, S. J. Davis, S. Thiel, and J. C. Jensenius. 2000. Distinct pathways of mannan-binding lectin (MBL)- and C1-complex autoactivation revealed by reconstitution of MBL with recombinant MBL-associated serine protease-2. J. Immunol. 165: 2093-2100.
21. Chen, C. B., and R. Wallis. 2004. Two mechanisms for mannose-binding protein modulation of the activity of its associated serine proteases. J. Biol. Chem. 279: 26058-26065.
22. Gal, P., V. Harmat, A. Kocsis, T. Bian, L. Barna, G. Ambrus, B. Vegh, J. Balczer, R. B. Sim, G. Naray-Szabo, and P. Zavodszky. 2005. A true autoactivating enzyme: structural insight into mannose-binding lectin-associated serine protease-2 activations. J. Biol. Chem. 280: 33435-33444.
23. Trouw, L. A., A. M. Blom, and P. Gasque. 2008. Role of complement and complement regulators in the removal of apoptotic cells. Mol. Immunol. 45: 1199-1207.
24. Harmat, V., P. Gal, J. Kardos, K. Szilagyi, G. Ambrus, B. Vegh, G. Naray-Szabo, and P. Zavodszky. 2004. The structure of MBL-associated serine protease-2 reveals that identical substrate specificities of C1s and MASP-2 are realized through different sets of enzyme-substrate interactions. J. Mol. Biol. 342: 1533-1546.
25. Rossi, V., F. Teillet, N. M. Thielens, I. Bally, and G. J. Arlaud. 2005. Functional characterization of complement proteases C1s/mannan-binding lectin-associated serine protease-2 (MASP-2) chimeras reveals the higher C4 recognition efficacy of the MASP-2 complement control protein modules. J. Biol. Chem. 280: 41811-41818.
26. Lozano, F., B. Suárez, A. Muñoz, J. C. Jensenius, J. Mensa, J. Vives, and J. P. Horcajada. 2005. Novel MASP2 variants detected among North African and Sub-Saharan individuals. Tissue Antigens 66: 131-135.
27. Rossi, V., S. Cseh, I. Bally, N. M. Thielens, J. C. Jensenius, and G. J. Arlaud. 2001. Substrate specificities of recombinant mannan-binding lectin-associated serine proteases-1 and -2. J. Biol. Chem. 276: 40880-40887.
28. Iwaki, D., and T. Fujita. 2005. Production and purification of recombinants of mouse MASP-2 and sMAP. J. Endotoxin Res. 11: 47-50.
29. Trombetta, E. S., and A. J. Parodi. 2003. Quality control and protein folding in the secretory pathway. Annu. Rev. Cell. Dev. Biol. 19: 649-676.
30. Feinberg, H., J. C. Uitdehaag, J. M. Davies, R. Wallis, K. Drickamer, and W. I. Weiss. 2003. Crystal structure of the CUB1-EGF-CUB2 region of mannose-binding protein associated serine protease-2. EMBO J. 22: 2348-2359.
31. Teillet, F., M. Lacroix, S. Thiel, D. Weilguny, T. Agger, G. J. Arlaud, and N. M. Thielens. 2007. Identification of the site of human mannan-binding lectin involved in the interaction with its partner serine proteases: the essential role of Lys55. J. Immunol. 178: 5710-5716.