528
Views
5
CrossRef citations to date
0
Altmetric
Original Article

Gain of function mutant of complement factor B K323E mimics pathogenic C3NeF autoantibodies in convertase assays

, , , , , & show all
Pages 18-24 | Received 14 Aug 2017, Accepted 29 Dec 2017, Published online: 08 Jan 2018

References

  • Ricklin D, Hajishengallis G, Yang K, et al. Complement: a key system for immune surveillance and homeostasis. Nat Immunol. 2010;11:785–797.
  • Fleming SD. Natural antibodies, autoantibodies and complement activation in tissue injury. Autoimmunity. 2006;39:379–386.
  • Schmidt CQ, Lambris JD, Ricklin D. Protection of host cells by complement regulators. Immunol Rev. 2016;274:152–171.
  • Kajander T, Lehtinen MJ, Hyvarinen S, et al. Dual interaction of factor H with C3d and glycosaminoglycans in host-nonhost discrimination by complement. Proc Natl Acad Sci USA. 2011;108:2897–2902.
  • Sethi S, Nester CM, Smith RJ. Membranoproliferative glomerulonephritis and C3 glomerulopathy: resolving the confusion. Kidney Int. 2012;81:434–441.
  • Zhang Y, Meyer NC, Wang K, et al. Causes of alternative pathway dysregulation in dense deposit disease. Clin J Am Soc Nephrol. 2012;7:265–274.
  • Servais A, Fremeaux-Bacchi V, Lequintrec M, et al. Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome. J Med Genet. 2007;44:193–199.
  • Seelen MA, Daha MR. The role of complement in autoimmune renal disease. Autoimmunity. 2006;39:411–415.
  • Daha MR, Austen KF, Fearon DT. Heterogeneity, polypeptide chain composition and antigenic reactivity of C3 nephritic factor. J Immunol. 1978;120:1389–1394.
  • Paixao-Cavalcante D, Lopez-Trascasa M, Skattum L, et al. Sensitive and specific assays for C3 nephritic factors clarify mechanisms underlying complement dysregulation. Kidney Int. 2012;82:1084–1092.
  • Daha MR, Van Es LA. Stabilization of homologous and heterologous cell-bound amplification convertases, C3bBb, by C3 nephritic factor. Immunology. 1981;43:33–38.
  • Jozsi M, Strobel S, Dahse HM, et al. Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome. Blood. 2007;110:1516–1518.
  • Goodship TH, Pappworth IY, Toth T, et al. Factor H autoantibodies in membranoproliferative glomerulonephritis. Mol Immunol. 2012;52:200–206.
  • Parente R, Clark SJ, Inforzato A, et al. Complement factor H in host defense and immune evasion. Cell Mol Life Sci. 2017;74:1605–1624.
  • Lopez-Trascasa M, Marin MA, Fontan G. C3 nephritic factor determination. A comparison between two methods. J Immunol Methods. 1987;98:77–82.
  • Prohaszka Z, Nilsson B, Frazer-Abel A, et al. Complement analysis 2016: clinical indications, laboratory diagnostics and quality control. Immunobiology. 2016;221:1247–1258.
  • Okroj M, Holmquist E, King BC, et al. Functional analyses of complement convertases using C3 and C5-depleted sera. PLoS One. 2012;7:e47245.
  • Blom AM, Volokhina EB, Fransson V, et al. A novel method for direct measurement of complement convertases activity in human serum. Clin Exp Immunol. 2014;178:142–153.
  • Harris CL, Pettigrew DM, Lea SM, et al. Decay-accelerating factor must bind both components of the complement alternative pathway C3 convertase to mediate efficient decay. J Immunol. 2007;178:352–359.
  • Goicoechea de Jorge E, Harris CL, Esparza-Gordillo J, et al. Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome. Proc Natl Acad Sci USA. 2007;104:240–245.
  • Matthews WJ Jr, Goldberger G, Marino JT Jr, et al. Complement proteins C2, C4 and factor B. Effect of glycosylation on their secretion and catabolism. Biochem J. 1982;204:839–846.
  • Goodship TH, Cook HT, Fakhouri F, et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) controversies conference. Kidney Int. 2017;91:539–551.
  • Leroy V, Fremeaux-Bacchi V, Peuchmaur M, et al. Membranoproliferative glomerulonephritis with C3NeF and genetic complement dysregulation. Pediatr Nephrol. 2011;26:419–424.
  • Schwertz R, Rother U, Anders D, et al. Complement analysis in children with idiopathic membranoproliferative glomerulonephritis: a long-term follow-up. Pediatr Allergy Immunol. 2001;12:166–172.
  • Spitzer RE, Stitzel AE, Tsokos GC. Evidence that production of autoantibody to the alternative pathway C3 convertase is a normal physiologic event. J Pediatr. 1990;116:S103–S108.
  • Spitzer RE, Stitzel AE, Tsokos GC. Study of the idiotypic response to autoantibody to the alternative pathway C3/C5 convertase in normal individuals, patients with membranoproliferative glomerulonephritis, and experimental animals. Clin Immunol Immunopathol. 1992;62:291–294.
  • Okroj M, Mark L, Stokowska A, et al. Characterization of the complement inhibitory function of rhesus rhadinovirus complement control protein (RCP). J Biol Chem. 2009;284:505–514.
  • Rother U. A new screening test for C3 nephritis factor based on a stable cell bound convertase on sheep erythrocytes. J Immunol Methods. 1982;51:101–107.
  • West CD. A hemolytic method for the measurement of nephritic factor. J Immunol Methods. 2008;335:1–7.
  • Van Dijk H, Rademaker PM, Willers JM. Determination of alternative pathway of complement activity in mouse serum using rabbit erythrocytes. J Immunol Methods. 1980;36:29–39.
  • Bergseth G, Ludviksen JK, Kirschfink M, et al. An international serum standard for application in assays to detect human complement activation products. Mol Immunol. 2013;56:232–239.
  • Liu C, Dalby B, Chen W, et al. Transient transfection factors for high-level recombinant protein production in suspension cultured mammalian cells. Mol Biotechnol. 2008;39:141–153.
  • Ido H, Harada K, Futaki S, et al. Molecular dissection of the alpha-dystroglycan- and integrin-binding sites within the globular domain of human laminin-10. J Biol Chem. 2004;279:10946–10954.
  • Smith RJ, Alexander J, Barlow PN, et al. and G. Dense Deposit Disease Focus. New approaches to the treatment of dense deposit disease. J Am Soc Nephrol. 2007;18:2447–2456.
  • Rudnicki M. Rituximab for treatment of membranoproliferative Glomerulonephritis and C3 Glomerulopathies. Biomed Res Int. 2017;2017:2180508.
  • Rousset-Rouviere C, Cailliez M, Garaix F, et al. Rituximab fails where eculizumab restores renal function in C3nef-related DDD. Pediatr Nephrol. 2014;29:1107–1111.
  • Giaime P, Daniel L, Burtey S. Remission of C3 glomerulopathy with rituximab as only immunosuppressive therapy. Clin Nephrol. 2015;83:57–60.
  • McCaughan JA, O'Rourke DM, Courtney AE. Recurrent dense deposit disease after renal transplantation: an emerging role for complementary therapies. Am J Transplant. 2012;12:1046–1051.
  • Nester CM, Smith RJ. Treatment options for C3 glomerulopathy. Curr Opin Nephrol Hypertens. 2013;22:231–237.
  • Noris M, Remuzzi G. Glomerular diseases dependent on complement activation, including atypical hemolytic uremic syndrome, membranoproliferative Glomerulonephritis, and C3 Glomerulopathy: core curriculum 2015. Am J Kidney Dis. 2015;66:359–375.
  • Marinozzi MC, Chauvet S, Le Quintrec M, et al. C5 nephritic factors drive the biological phenotype of C3 glomerulopathies. Kidney Int. 2017;92:1232–1241.

Reprints and Corporate Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

To request a reprint or corporate permissions for this article, please click on the relevant link below:

Academic Permissions

Please note: Selecting permissions does not provide access to the full text of the article, please see our help page How do I view content?

Obtain permissions instantly via Rightslink by clicking on the button below:

If you are unable to obtain permissions via Rightslink, please complete and submit this Permissions form. For more information, please visit our Permissions help page.