C3 Glomerulopathy

Abrera-Abeleda MA, Nishimura C, Frees K, Jones M, Maga T, Katz LM, Zhang Y, Smith RJH. Allele variants of complement genes associated with dense deposit disease. J Am Soc Nephrol. 2011;22:1551–9. [PMC free article: PMC3148710] [PubMed: 21784901]

Abrera-Abeleda MA, Nishimura C, Smith JL, Sethi S, McRae JL, Murphy BF, Silvestri G, Skerka C, Józsi M, Zipfel PF, Hageman GS, Smith RJ. Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with membranoproliferative glomerulonephritis type II (dense deposit disease). J Med Genet. 2006;43:582–9. [PMC free article: PMC2564553] [PubMed: 16299065]

Alberts B, Johnson A, Lewis J, et al. Studying gene expression and function. In: Molecular Biology of the Cell. 4 ed. New York: Garland Science. Available online. 2002. Accessed 2-23-22.

Angelo JR, Bell CS, Braun MC. Allograft failure in kidney transplant recipients with membranoproliferative glomerulonephritis. Am J Kidney Dis. 2011;57:291–9. [PubMed: 21215503]

Appel GB, Cook HT, Hageman G, Jennette JC, Kashgarian M, Kirschfink M, Lambris JD, Lanning L, Lutz HU, Meri S, Rose NR, Salant DJ, Sethi S, Smith RJ, Smoyer W, Tully HF, Tully SP, Walker P, Welsh M, Wurzner R, Zipfel PF. Membranoproliferative glomerulonephritis type II (dense deposit disease): an update. J Am Soc Nephrol. 2005;16:1392–403. [PubMed: 15800116]

Athanasiou Y, Voskarides K, Gale DP, Damianou L, Patsias C, Zavros M, Maxwell PH, Cook HT, Demosthenous P, Hadjisavvas A, Kyriacou K, Zouvani I, Pierides A, Deltas C. Familial C3 glomerulopathy associated with CFHR5 mutations: clinical characteristics of 91 patients in 16 pedigrees. Clin J Am Soc Nephrol. 2011;6:1436–46. [PMC free article: PMC3109942] [PubMed: 21566112]

Ault BH, Schmidt BZ, Fowler NL, Kashtan CE, Ahmed AE, Vogt BA, Colten HR. Human factor H deficiency. Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism. J Biol Chem. 1997;272:25168–75. [PubMed: 9312129]

Azukaitis K, Simkova E, Majid MA, Galiano M, Benz K, Amann K, Bockmeyer C, Gajjar R, Meyers KE, Cheong HI, Lange-Sperandio B, Jungraithmayr T, Fremeaux-Bacchi V, Bergmann C, Bereczki C, Miklaszewska M, Csuka D, Prohászka Z, Gipson P, Sampson MG, Lemaire M, Schaefer F. The phenotypic spectrum of nephropathies associated with mutations in diacylglycerol kinase ε. J Am Soc Nephrol. 2017;28:3066–75. [PMC free article: PMC5619969] [PubMed: 28526779]

Besbas N, Gulhan B, Gucer S, Korkmaz E, Ozaltin F. A novel CFHR5 mutation associated with C3 glomerulonephritis in a Turkish girl. J Nephrol. 2014;27:457–60. [PubMed: 24536001]

Blanc C, Togarsimalemath SK, Chauvet S, Le Quintrec M, Moulin B, Buchler M, Jokiranta TS, Roumenina LT, Fremeaux-Bacchi V, Dragon-Durey MA. Anti-factor H autoantibodies in C3 glomerulopathies and in atypical hemolytic uremic syndrome: one target, two diseases. J Immunol. 2015;194:5129–38. [PubMed: 25917093]

Bomback AS, Smith RJ, Barile GR, Zhang Y, Heher EC, Herlitz L, Stokes MB, Markowitz GS, D'Agati VD, Canetta PA, Radhakrishnan J, Appel GB. Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol. 2012;7:748–56. [PMC free article: PMC3338285] [PubMed: 22403278]

Bu F, Borsa NG, Jones MB, Takanami E, Nishimura C, Hauer JJ, Azaiez H, Black-Ziegelbein EA, Meyer NC, Kolbe DL, Li Y, Frees K, Schnieders MJ, Thomas C, Nester C, Smith RJ. High-throughput genetic testing for thrombotic microangiopathies and C3 glomerulopathies. J Am Soc Nephrol. 2016;27:1245–53. [PMC free article: PMC4814193] [PubMed: 26283675]

Cameron JS, Turner DR, Heaton J, Williams DG, Ogg CS, Chantler C, Haycock GB, Hicks J. Idiopathic mesangiocapillary glomerulonephritis. Comparison of types I and II in children and adults and long-term prognosis. Am J Med. 1983;74:175–92. [PubMed: 6337487]

Cebeci Z, Bayraktar S, Oray M, Kir N. Multimodal imaging of membranoproliferative glomerulonephritis type II. Saudi J Ophthalmol. 2016;30:260–3. [PMC free article: PMC5161817] [PubMed: 28003788]

Chauvet S, Fremeaux-Bacchi V, Petitprez F, Karras A, Daniel L, Burtey S, Choukroun G, Delmas Y, Guerrot D, François A, Le Quintrec M, Javaugue V, Ribes D, Vrigneaud L, Arnulf B, Goujon JM, Ronco P, Touchard G, Bridoux F. Treatment of B-cell disorder improves renal outcome of patients with monoclonal gammopathy-associated C3 glomerulopathy. Blood. 2017;129:1437–47. [PubMed: 28069603]

Chen Q, Muller D, Rudolph B, Hartmann A, Kuwertz-Broking E, Wu K, Kirschfink M, Skerka C, Zipfel PF. Combined C3b and factor B autoantibodies and MPGN type II. N Engl J Med. 2011;365:2340–2. [PubMed: 22168663]

Chen Q, Wiesener M, Eberhardt HU, Hartmann A, Uzonyi B, Kirschfink M, Amann K, Buettner M, Goodship T, Hugo C, Skerka C, Zipfel PF. Complement factor H-related hybrid protein deregulates complement in dense deposit disease. J Clin Invest. 2014;124:145–55. [PMC free article: PMC3871254] [PubMed: 24334459]

D'Agati VD, Bomback AS. C3 glomerulopathy: what's in a name? Kidney Int. 2012;82:379–81. [PubMed: 22846813]

Daina E, Noris M, Remuzzi G. Eculizumab in a patient with dense-deposit disease. N Engl J Med. 2012;366:1161–3. [PubMed: 22435382]

Dalvin LA, Fervenza FC, Sethi S, Pulido JS. Shedding light on fundus drusen associated with membranoproliferative glomerulonephritis: breaking stereotypes of types I, II, and III. Retin Cases Brief Rep. 2016;10:72–8. [PubMed: 26110522]

Deltas C, Gale D, Cook T, Voskarides K, Athanasiou Y, Pierides A. C3 glomerulonephritis/CFHR5 nephropathy is an endemic disease in Cyprus: clinical and molecular findings in 21 families. Adv Exp Med Biol. 2013;735:189–96. [PubMed: 23402027]

Dragon-Durey MA, Fremeaux-Bacchi V, Loirat C, Blouin J, Niaudet P, Deschenes G, Coppo P, Herman Fridman W, Weiss L. Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases. J Am Soc Nephrol. 2004;15:787–95. [PubMed: 14978182]

D'Souza YB, Jones CJ, Short CD, Roberts IS, Bonshek RE. Oligosaccharide composition is similar in drusen and dense deposits in membranoproliferative glomerulonephritis type II. Kidney Int. 2009;75:824–7. [PubMed: 19177159]

Eisinger AJ, Shortland JR, Moorhead PJ. Renal disease in partial lipodystrophy. Q J Med. 1972;41:343–54. [PubMed: 4561162]

Fang CJ, Fremeaux-Bacchi V, Liszewski MK, Pianetti G, Noris M, Goodship TH, Atkinson JP. Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome. Blood. 2008;111:624–32. [PMC free article: PMC2200836] [PubMed: 17914026]

Fremeaux-Bacchi V, Fakhouri F, Garnier A, Bienaimé F, Dragon-Durey MA, Ngo S, Moulin B, Servais A, Provot F, Rostaing L, Burtey S, Niaudet P, Deschênes G, Lebranchu Y, Zuber J, Loirat C. Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol. 2013;8:554–62. [PMC free article: PMC3613948] [PubMed: 23307876]

Fremeaux-Bacchi V, Fakhouri F, Loirat C. Hemolytic-uremic syndrome: what is the mechanism? Rev Prat. 2008;58:2093–6. [PubMed: 19213534]

Fremeaux-Bacchi V, Weiss L, Brun P, Kazatchkine MD. Selective disappearance of C3NeF IgG autoantibody in the plasma of a patient with membranoproliferative glomerulonephritis following renal transplantation. Nephrol Dial Transplant. 1994;9:811–4. [PubMed: 7970124]

Fujita T, Nozu K, Iijima K, Kamioka I, Kaito H, Tanaka R, Nakanishi K, Matsuo M, Yoshikawa N. Long-term follow-up of juvenile acute nonproliferative glomerulitis (JANG). Pediatr Nephrol. 2007;22:1957–61. [PubMed: 17674054]

Gale DP, de Jorge EG, Cook HT, Martínez-Barricarte R, Hadjisavvas A, McLean AG, Pusey CD, Pierides A, Kyriacou K, Athanasiou Y, Voskarides K, Deltas C, Palmer A, Fremeaux-Bacchi V, de Cordoba SR, Maxwell PH, Pickering MC. Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis. Lancet. 2010;376:794–801. [PMC free article: PMC2935536] [PubMed: 20800271]

Gale DP, Owen-Casey M. Membranoproliferative glomerulonephritis and C3 glomerulopathy. In: Harber M, ed. Practical Nephrology. London: Springer; 2014:189-202.

Goicoechea de Jorge E, Gale DP, Cook HT, Martínez-Barricate A, Hadjisavvas A, Pusey CD, Palmer A, Fremeaux-Bacchi V, Rodriguez de Cordoba S, Maxwell PH, Pickering MC. A mutant complement factor H-related 5 protein is associated with familial C3 glomerulonephritis. Mol Immunol. 2009;46:2822.

Gold B, Merriam JE, Zernant J, Hancox LS, Taiber AJ, Gehrs K, Cramer K, Neel J, Bergeron J, Barile GR, Smith RT, Hageman GS, Dean M, Allikmets R, et al. Variation in factor B (BF) and complement component 2 (C2) genes is associated with age-related macular degeneration. Nat Genet. 2006;38:458–62. [PMC free article: PMC2921703] [PubMed: 16518403]

Goodship TH, Cook HT, Fakhouri F, Fervenza FC, Fremeaux-Bacchi V, Kavanagh D, Nester CM, Noris M, Pickering MC, Rodríguez de Córdoba S, Roumenina LT, Sethi S, Smith RJ, et al. Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference. Kidney Int. 2017;91:539–51. [PubMed: 27989322]

Habib R, Gubler MC, Loirat C, Maiz HB, Levy M. Dense deposit disease: a variant of membranoproliferative glomerulonephritis. Kidney Int. 1975;7:204–15. [PubMed: 1095806]

Hageman GS, Anderson DH, Johnson LV, Hancox LS, Taiber AJ, Hardisty LI, Hageman JL, Stockman HA, Borchardt JD, Gehrs KM, Smith RJ, Silvestri G, Russell SR, Klaver CC, Barbazetto I, Chang S, Yannuzzi LA, Barile GR, Merriam JC, Smith RT, Olsh AK, Bergeron J, Zernant J, Merriam JE, Gold B, Dean M, Allikmets R. A common haplotype in the complement regulatory gene factor H (HF1/CFH) predisposes individuals to age-related macular degeneration. Proc Natl Acad Sci U S A. 2005;102:7227–32. [PMC free article: PMC1088171] [PubMed: 15870199]

Hulleman JD. Malattia Leventinese/Doyne honeycomb retinal dystrophy: similarities to age-related macular degeneration and potential therapies. Adv Exp Med Biol. 2016;854:153–8. [PubMed: 26427406]

Iatropoulos P, Noris M, Mele C, Piras R, Valoti E, Bresin E, Curreri M, Mondo E, Zito A, Gamba S, Bettoni S, Murer L, Fremeaux-Bacchi V, Vivarelli M, Emma F, Daina E, Remuzzi G. Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome. Mol Immunol. 2016;71:131–42. [PubMed: 26895476]

Imamura H, Konomoto T, Tanaka E, Hisano S, Yoshida Y, Fujimura Y, Miyata T, Nunoi H. Familial C3 glomerulonephritis associated with mutations in the gene for complement factor B. Nephrol Dial Transplant. 2015;30:862–4. [PubMed: 25758434]

Johnson SA, Wong EK, Taylor CM. Making sense of the spectrum of glomerular disease associated with complement dysregulation. Pediatr Nephrol. 2014;29:1883–94. [PubMed: 23852337]

Kambham N. Postinfectious glomerulonephritis. Adv Anat Pathol. 2012;19:338–47. [PubMed: 22885383]

Khalighi MA, Wang S, Henriksen KJ, Bock M, Keswani M, Meehan SM, Chang A. Revisiting post-infectious glomerulonephritis in the emerging era of C3 glomerulopathy. Clin Kidney J. 2016;9:397–402. [PMC free article: PMC4886922] [PubMed: 27274823]

Kobayashi Y, Yang S, Nykamp K, Garcia J, Lincoln SE, Topper SE. Pathogenic variant burden in the ExAC database: an empirical approach to evaluating population data for clinical variant interpretation. Genome Med. 2017;9:13. [PMC free article: PMC5295186] [PubMed: 28166811]

Kurtz KA, Schlueter AJ. Management of membranoproliferative glomerulonephritis type II with plasmapheresis. J Clin Apher. 2002;17:135–7. [PubMed: 12378549]

Lemaire M, Fremeaux-Bacchi V, Schaefer F, Choi M, Tang WH, Le Quintrec M, Fakhouri F, Taque S, Nobili F, Martínez F, Ji W, Overton JD, Mane SM, Nürnberg G, Altmüller J, Thiele H, Morin D, Deschenes G, Baudouin V, Llanas B, Collard L, Majid MA, Simkova E, Nürnberg P, Rioux-Leclerc N, Moeckel GW, Gubler MC, Hwa J, Loirat C, Lifton RP. Recessive mutations in DGKE cause atypical hemolytic-uremic syndrome. Nat Genet. 2013;45:531–6. [PMC free article: PMC3719402] [PubMed: 23542698]

Licht C, Fremeaux-Bacchi V. Hereditary and acquired complement dysregulation in membranoproliferative glomerulonephritis. Thromb Haemost. 2009;101:271–8. [PubMed: 19190809]

Licht C, Heinen S, Jozsi M, Loschmann I, Saunders RE, Perkins SJ, Skerka C, Kirschfink M, Hoppe B, Zipfel PF. Deletion of Lys224 in regulatory domain 4 of factor H reveals a novel pathomechanism for dense deposit disease (MPGNII). Kidney Int. 2006;70:42–50. [PubMed: 16612335]

Licht C, Mengel M. Membranoproliferative glomerulonephritis. In: Geary DF, Schaefer F, eds. Comprehensive Pediatric Nephrology. 1 ed. Philadelphia, PA: Mosby; 2008:269-89.

Lu DF, McCarthy AM, Lanning LD, Delaney C, Porter C. A descriptive study of individuals with membranoproliferative glomerulonephritis. Nephrol Nurs J. 2007;34:295–302. [PubMed: 17644874]

Lu DF, Moon M, Lanning LD, McCarthy AM, Smith RJ. Clinical features and outcomes of 98 children and adults with dense deposit disease. Pediatr Nephrol. 2012;27:773–81. [PMC free article: PMC4423603] [PubMed: 22105967]

Malik TH, Lavin PJ, Goicoechea de Jorge E, Vernon KA, Rose KL, Patel MP, de Leeuw M, Neary JJ, Conlon PJ, Winn MP, Pickering MC. A hybrid CFHR3-1 gene causes familial C3 glomerulopathy. J Am Soc Nephrol. 2012;23:1155–60. [PMC free article: PMC3380655] [PubMed: 22626820]

Marks SD, Rees L. Spontaneous clinical improvement in dense deposit disease. Pediatr Nephrol. 2000;14:322–4. [PubMed: 10775078]

Martínez-Barricarte R, Heurich M, Valdes-Cañedo F, Vazquez-Martul E, Torreira E, Montes T, Tortajada A, Pinto S, Lopez-Trascasa M, Morgan BP, Llorca O, Harris CL, Rodríguez de Córdoba S. Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation. J Clin Invest. 2010;120:3702–12. [PMC free article: PMC2947238] [PubMed: 20852386]

Mathieson PW, Peters DK. Lipodystrophy in MCGN type II: the clue to links between the adipocyte and the complement system. Nephrol Dial Transplant. 1997;12:1804–6. [PubMed: 9306323]

McCaughan JA, O'Rourke DM, Courtney AE. Recurrent dense deposit disease after renal transplantation: an emerging role for complementary therapies. Am J Transplant. 2012;12:1046–51. [PubMed: 22233157]

McRae JL, Cowan PJ, Power DA, Mitchelhill KI, Kemp BE, Morgan BP, Murphy BF. Human factor H-related protein 5 (FHR-5). A new complement-associated protein. J Biol Chem. 2001;276:6747–54. [PubMed: 11058592]

McRae JL, Duthy TG, Griggs KM, Ormsby RJ, Cowan PJ, Cromer BA, McKinstry WJ, Parker MW, Murphy BF, Gordon DL. Human factor H-related protein 5 has cofactor activity, inhibits C3 convertase activity, binds heparin and C-reactive protein, and associates with lipoprotein. J Immunol. 2005;174:6250–6. [PubMed: 15879123]

Medjeral-Thomas N, Malik TH, Patel MP, Toth T, Cook HT, Tomson C, Pickering MC. A novel CFHR5 fusion protein causes C3 glomerulopathy in a family without Cypriot ancestry. Kidney Int. 2014;85:933–7. [PMC free article: PMC3789233] [PubMed: 24067434]

Merinero HM, García SP, García-Fernández J, Arjona E, Tortajada A, Rodríguez de Córdoba S. Complete functional characterization of disease-associated genetic variants in the complement factor H gene. Kidney Int. 2018;93:470–81. [PubMed: 28941939]

Mullins RF, Aptsiauri N, Hageman GS. Structure and composition of drusen associated with glomerulonephritis: implications for the role of complement activation in drusen biogenesis. Eye (Lond). 2001;15:390–5. [PubMed: 11450763]

Murphy B, Georgiou T, Machet D, Hill P, McRae J. Factor H-related protein-5: a novel component of human glomerular immune deposits. Am J Kidney Dis. 2002;39:24–7. [PubMed: 11774097]

Nasr SH, Valeri AM, Appel GB, Sherwinter J, Stokes MB, Said SM, Markowitz GS, D'Agati VD. Dense deposit disease: clinicopathologic study of 32 pediatric and adult patients. Clin J Am Soc Nephrol. 2009;4:22–32. [PMC free article: PMC2615696] [PubMed: 18971369]

Nava J, Moran S, Figueroa V, Salinas A, Lopez M, Urbina R, Gutierrez A, Lujan JL, Orozco A, Montufar R, Piccoli GB. Successful pregnancy in a CKD patient on a low-protein, supplemented diet: an opportunity to reflect on CKD and pregnancy in Mexico, an emerging country. J Nephrol. 2017;30:877–82. [PubMed: 28918595]

Nester CM, Smith RJ. Complement inhibition in C3 glomerulopathy. Semin Immunol. 2016;28:241–9. [PubMed: 27402056]

Nester CM, Smith RJ. Diagnosis and treatment of C3 glomerulopathy. Clin Nephrol. 2013a;80:395–403. [PubMed: 23993166]

Nicolas C, Vuiblet V, Baudouin V, Macher MA, Vrillon I, Biebuyck-Gouge N, Dehennault M, Gié S, Morin D, Nivet H, Nobili F, Ulinski T, Ranchin B, Marinozzi MC, Ngo S, Fremeaux-Bacchi V, Pietrement C. C3 nephritic factor associated with C3 glomerulopathy in children. Pediatr Nephrol. 2014;29:85–94. [PubMed: 24068526]

Nilsson SC, Kalchishkova N, Trouw LA, Fremeaux-Bacchi V, Villoutreix BO, Blom AM. Mutations in complement factor I as found in atypical hemolytic uremic syndrome lead to either altered secretion or altered function of factor I. Eur J Immunol. 2010;40:172–85. [PubMed: 19877009]

Noris M, Remuzzi G. Genetics of immune-mediated glomerular diseases: focus on complement. Semin Nephrol. 2017;37:447–63. [PubMed: 28863792]

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;66:359–75. [PMC free article: PMC4528072] [PubMed: 26032627]

Ohi H, Watanabe S, Fujita T, Yasugi T. Significance of C3 nephritic factor (C3NeF) in non-hypocomplementaemic serum with membranoproliferative glomerulonephritis (MPGN). Clin Exp Immunol. 1992;89:479–84. [PMC free article: PMC1554488] [PubMed: 1516262]

Osborne AJ, Breno M, Borsa NG, Bu F, Fremeaux-Bacchi V, Gale DP, van den Heuvel LP, Kavanagh D, Noris M, Pinto S, Rallapalli PM, Remuzzi G, Rodríguez de Cordoba S, Ruiz A, Smith RJH, Vieira-Martins P, Volokhina E, Wilson V, Goodship THJ, Perkins SJ. Statistical validation of rare complement variants provides insights into the molecular basis of atypical hemolytic uremic syndrome and C3 glomerulopathy. J Immunol. 2018;2018;200:2464–78. [PMC free article: PMC6324840] [PubMed: 29500241]

Ossoli A, Lucca F, Boscutti G, Remaley AT, Calabresi L. Familial LCAT deficiency: from pathology to enzyme replacement therapy. Clinical Lipidology. 2015;10:405–13.

Ozaltin F, Li B, Rauhauser A, An SW, Soylemezoglu O, Gonul II, Taskiran EZ, Ibsirlioglu T, Korkmaz E, Bilginer Y, Duzova A, Ozen S, Topaloglu R, Besbas N, Ashraf S, Du Y, Liang C, Chen P, Lu D, Vadnagara K, Arbuckle S, Lewis D, Wakeland B, Quigg RJ, Ransom RF, Wakeland EK, Topham MK, Bazan NG, Mohan C, Hildebrandt F, Bakkaloglu A, Huang CL, Attanasio M. DGKE variants cause a glomerular microangiopathy that mimics membranoproliferative GN. J Am Soc Nephrol. 2013;24:377–84. [PMC free article: PMC3582208] [PubMed: 23274426]

Paixão-Cavalcante D, López-Trascasa M, Skattum L, Giclas PC, Goodship TH, de Córdoba SR, Truedsson L, Morgan BP, Harris CL. Sensitive and specific assays for C3 nephritic factors clarify mechanisms underlying complement dysregulation. Kidney Int. 2012;82:1084–92. [PMC free article: PMC3608896] [PubMed: 22854646]

Piccoli GB, Alrukhaimi M, Liu ZH, Zakharova E, Levin A, et al. What we do and do not know about women and kidney diseases; questions unanswered and answers unquestioned: reflection on World Kidney Day and International Woman's Day. BMC Nephrol. 2018;19:66. [PMC free article: PMC5856379] [PubMed: 29544451]

Pickering MC, D'Agati VD, Nester CM, Smith RJ, Haas M, Appel GB, Alpers CE, Bajema IM, Bedrosian C, Braun M, Doyle M, Fakhouri F, Fervenza FC, Fogo AB, Fremeaux-Bacchi V, Gale DP, Goicoechea de Jorge E, Griffin G, Harris CL, Holers VM, Johnson S, Lavin PJ, Medjeral-Thomas N, Paul Morgan B, Nast CC, Noel LH, Peters DK, Rodríguez de Córdoba S, Servais A, Sethi S, Song WC, Tamburini P, Thurman JM, Zavros M, Cook HT. C3 glomerulopathy: consensus report. Kidney Int. 2013;84:1079–89. [PMC free article: PMC3842953] [PubMed: 24172683]

Rabasco C, Cavero T, Román E, Rojas-Rivera J, Olea T, Espinosa M, Cabello V, Fernández-Juarez G, González F, Ávila A, Baltar JM, Díaz M, Alegre R, Elías S, Antón M, Frutos MA, Pobes A, Blasco M, Martín F, Bernis C, Macías M, Barroso S, de Lorenzo A, Ariceta G, López-Mendoza M, Rivas B, López-Revuelta K, Campistol JM, Mendizábal S, de Córdoba SR, Praga M, et al. Effectiveness of mycophenolate mofetil in C3 glomerulonephritis. Kidney Int. 2015;88:1153–60. [PubMed: 26221755]

Recalde S, Tortajada A, Subias M, Anter J, Blasco M, Maranta R, Coco R, Pinto S, Noris M, García-Layana A, Rodríguez de Córdoba S. Molecular basis of factor H R1210C association with ocular and renal diseases. J Am Soc Nephrol. 2016;27:1305–11. [PMC free article: PMC4849834] [PubMed: 26376859]

Riedl M, Thorner P, Licht C. C3 glomerulopathy. Pediatr Nephrol. 2017;32:43–57. [PubMed: 27056062]

Rodríguez de Córdoba S, Esparza-Gordillo J, Goicoechea de Jorge E, Lopez-Trascasa M, Sanchez-Corral P. The human complement factor H: functional roles, genetic variations and disease associations. Mol Immunol. 2004;41:355–67. [PubMed: 15163532]

Salvadori M, Bertoni E. Complement related kidney diseases: recurrence after transplantation. World J Transplant. 2016;6:632–45. [PMC free article: PMC5175220] [PubMed: 28058212]

Savige J, Amos L, Ierino F, Mack HG, Symons RC, Hughes P, Nicholls K, Colville D. Retinal disease in the C3 glomerulopathies and the risk of impaired vision. Ophthalmic Genet. 2016;37:369–76. [PubMed: 26915021]

Schwertz R, Rother U, Anders D, Gretz N, Scharer K, Kirschfink M. Complement analysis in children with idiopathic membranoproliferative glomerulonephritis: a long-term follow-up. Pediatr Allergy Immunol. 2001;12:166–72. [PubMed: 11473682]

Servais A, Fremeaux-Bacchi V, Lequintrec M, Salomon R, Blouin J, Knebelmann B, Grünfeld JP, Lesavre P, Noël LH, Fakhouri F. Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome. J Med Genet. 2007;44:193–9. [PMC free article: PMC2598029] [PubMed: 17018561]

Servais A, Noël LH, Fremeaux-Bacchi V, Lesavre P. C3 glomerulopathy. Contrib Nephrol. 2013;181:185–93. [PubMed: 23689580]

Servais A, Noel LH, Roumenina LT, Le Quintrec M, Ngo S, Dragon-Durey MA, Macher MA, Zuber J, Karras A, Provot F, Moulin B, Grünfeld JP, Niaudet P, Lesavre P, Fremeaux-Bacchi V. Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int. 2012;82:454–64. [PubMed: 22456601]

Sessa A, Battini G, Meroni M, Daidone G, Carnera I, Brambilla PL, Vigano G, Giordano F, Pallotti F, Torri Tarelli L, Calabresi L, Rolleri M, Bertolini S. Hypocomplementemic type II membranoproliferative glomerulonephritis in a male patient with familial lecithin-cholesterol acyltransferase deficiency due to two different allelic mutations. Nephron. 2001;88:268–72. [PubMed: 11423760]

Sethi S, Fervenza FC. Membranoproliferative glomerulonephritis: pathogenetic heterogeneity and proposal for a new classification. Semin Nephrol. 2011;31:341–8. [PubMed: 21839367]

Sethi S, Fervenza FC, Zhang Y, Zand L, Meyer NC, Borsa N, Nasr SH, Smith RJ. Atypical post-infectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement. Kidney Int. 2013;83:293–9. [PMC free article: PMC3561505] [PubMed: 23235567]

Sethi S, Fervenza FC, Zhang Y, Zand L, Vrana JA, Nasr SH, Theis JD, Dogan A, Smith RJ. C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int. 2012a;82:465–73. [PMC free article: PMC4438675] [PubMed: 22673887]

Sethi S, Haas M, Markowitz GS, D'Agati VD, Rennke HG, Jennette JC, Bajema IM, Alpers CE, Chang A, Cornell LD, Cosio FG, Fogo AB, Glassock RJ, Hariharan S, Kambham N, Lager DJ, Leung N, Mengel M, Nath KA, Roberts IS, Rovin BH, Seshan SV, Smith RJ, Walker PD, Winearls CG, Appel GB, Alexander MP, Cattran DC, Casado CA, Cook HT, De Vriese AS, Radhakrishnan J, Racusen LC, Ronco P, Fervenza FC. Mayo Clinic/Renal Pathology Society Consensus Report on Pathologic Classification, Diagnosis, and Reporting of GN. J Am Soc Nephrol. 2016;27:1278–87. [PMC free article: PMC4849835] [PubMed: 26567243]

Sethi S, Nester CM, Smith RJ. Membranoproliferative glomerulonephritis and C3 glomerulopathy: resolving the confusion. Kidney Int. 2012b;81:434–41. [PMC free article: PMC4428602] [PubMed: 22157657]

Smith RJ, Alexander J, Barlow PN, Botto M, Cassavant TL, Cook HT, de Cordoba SR, Hageman GS, Jokiranta TS, Kimberling WJ, Lambris JD, Lanning LD, Levidiotis V, Licht C, Lutz HU, Meri S, Pickering MC, Quigg RJ, Rops AL, Salant DJ, Sethi S, Thurman JM, Tully HF, Tully SP, van der Vlag J, Walker PD, Wurzner R, Zipfel PF. New approaches to the treatment of dense deposit disease. J Am Soc Nephrol. 2007;18:2447–56. [PMC free article: PMC4853920] [PubMed: 17675665]

Sohn EH, Wang K, Thompson S, Riker MJ, Hoffmann JM, Stone EM, Mullins RF. Comparison of drusen and modifying genes in autosomal dominant radial drusen and age-related macular degeneration. Retina. 2015;35:48–57. [PMC free article: PMC5513174] [PubMed: 25077532]

Sotsiou F. Postinfectious glomerulonephritis. Nephrol Dial Transplant. 2001;16 Suppl 6:68–70. [PubMed: 11568248]

Spitzer RE, Stitzel AE. Loss of autoantibody activity by alteration in autoantigen. Clin Immunol Immunopathol. 1996;80:211–3. [PubMed: 8764567]

Stone EM, Lotery AJ, Munier FL, Heon E, Piguet B, Guymer RH, Vandenburgh K, Cousin P, Nishimura D, Swiderski RE, Silvestri G, Mackey DA, Hageman GS, Bird AC, Sheffield VC, Schorderet DF. A single EFEMP1 mutation associated with both Malattia Leventinese and Doyne honeycomb retinal dystrophy. Nat Genet. 1999;22:199–202. [PubMed: 10369267]

Strobel S, Zimmering M, Papp K, Prechl J, Józsi M. Anti-factor B autoantibody in dense deposit disease. Mol Immunol. 2010;47:1476–83. [PubMed: 20193965]

Thomas S, Ranganathan D, Francis L, Madhan K, John GT. Current concepts in C3 glomerulopathy. Indian J Nephrol. 2014;24:339–48. [PMC free article: PMC4244712] [PubMed: 25484526]

Togarsimalemath SK, Sethi SK, Duggal R, Quintrec ML, Jha P, Daniel R, Gonnet F, Bansal S, Roumenina LT, Fremeaux-Bacchi V, Kher V, Dragon-Durey MA. A novel CFHR1-CFHR5 hybrid leads to a familial dominant C3 glomerulopathy. Kidney Int. 2017;92:876–87. [PubMed: 28729035]

Tortajada A, Yébenes H, Abarrategui-Garrido C, Anter J, García-Fernández JM, Martínez-Barricarte R, Alba-Domínguez M, Malik TH, Bedoya R, Cabrera Pérez R, López Trascasa M, Pickering MC, Harris CL, Sánchez-Corral P, Llorca O, Rodríguez de Córdoba S. C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. J Clin Invest. 2013;123:2434–46. [PMC free article: PMC3668852] [PubMed: 23728178]

Vaclavik V, Munier FL. Malattia Leventinese (autosomal dominant drusen). In: Querques G, Souied E, eds. Macular Dystrophies. Cham, Switzerland: Springer; 2016:39-51.

Vernon KA, Goicoechea de Jorge E, Hall AE, Fremeaux-Bacchi V, Aitman TJ, Cook HT, Hangartner R, Koziell A, Pickering MC. Acute presentation and persistent glomerulonephritis following streptococcal infection in a patient with heterozygous complement factor H-related protein 5 deficiency. Am J Kidney Dis. 2012;60:121–5. [PMC free article: PMC3382710] [PubMed: 22503529]

Vivarelli M, Emma F. Treatment of C3 glomerulopathy with complement blockers. Semin Thromb Hemost. 2014;40:472–7. [PubMed: 24799307]

Walker PD, Ferrario F, Joh K, Bonsib SM. Dense deposit disease is not a membranoproliferative glomerulonephritis. Mod Pathol. 2007;20:605–16. [PubMed: 17396142]

West CD, McAdams AJ, Witte DP. Acute non-proliferative glomerulitis: a cause of renal failure unique to children. Pediatr Nephrol. 2000;14:786–93. [PubMed: 10955928]

Westland R, Bodria M, Carrea A, Lata S, Scolari F, Fremeaux-Bacchi V, D'Agati VD, Lifton RP, Gharavi AG, Ghiggeri GM, Sanna-Cherchi S. Phenotypic expansion of DGKE-associated diseases. J Am Soc Nephrol. 2014;25:1408–14. [PMC free article: PMC4073436] [PubMed: 24511134]

Xiao X, Ghossein C, Tortajada A, Zhang Y, Meyer N, Jones M, Borsa NG, Nester CM, Thomas CP, de Córdoba SR, Smith RJ. Familial C3 glomerulonephritis caused by a novel CFHR5-CFHR2 fusion gene. Mol Immunol. 2016;77:89–96. [PubMed: 27490940]

Xiao X, Pickering MC, Smith RJH. C3 glomerulopathy: the genetic and clinical findings in dense deposit disease and C3 glomerulonephritis. Semin Thromb Hemost. 2014;40:465–71. [PubMed: 24799308]

Zand L, Lorenz EC, Cosio FG, Fervenza FC, Nasr SH, Gandhi MJ, Smith RJ, Sethi S. Clinical findings, pathology, and outcomes of C3GN after kidney transplantation. J Am Soc Nephrol. 2014;25:1110–7. [PMC free article: PMC4005307] [PubMed: 24357668]

Zhang Y, Meyer NC, Fervenza FC, Lau W, Keenan A, Cara-Fuentes G, Shao D, Akber A, Fremeaux-Bacchi V, Sethi S, Nester CM, Smith RJ. C4 nephritic factors in C3 glomerulopathy: a case series. Am J Kidney Dis. 2017;70:834–43. [PMC free article: PMC5701858] [PubMed: 28838767]

Zhang Y, Meyer NC, Wang K, Nishimura C, Frees K, Jones M, Katz LM, Sethi S, Smith RJ. Causes of alternative pathway dysregulation in dense deposit disease. Clin J Am Soc Nephrol. 2012;7:265–74. [PMC free article: PMC3280037] [PubMed: 22223606]

Zhang Y, Nester CM, Holanda DG, Marsh HC, Hammond RA, Thomas LJ, Meyer NC, Hunsicker LG, Sethi S, Smith RJ. Soluble CR1 therapy improves complement regulation in C3 glomerulopathy. J Am Soc Nephrol. 2013;24:1820–9. [PMC free article: PMC3810083] [PubMed: 23907509]

Zhu J, Chaki M, Lu D, Ren C, Wang SS, Rauhauser A, Li B, Zimmerman S, Jun B, Du Y, Vadnagara K, Wang H, Elhadi S, Quigg RJ, Topham MK, Mohan C, Ozaltin F, Zhou XJ, Marciano DK, Bazan NG, Attanasio M. Loss of diacylglycerol kinase epsilon in mice causes endothelial distress and impairs glomerular Cox-2 and PGE2 production. Am J Physiol Renal Physiol. 2016;310:F895–908. [PMC free article: PMC4867310] [PubMed: 26887830]

Zipfel PF, Skerka C, Chen Q, Wiech T, Goodship T, Johnson S, Fremeaux-Bacchi V, Nester C, de Córdoba SR, Noris M, Pickering M, Smith R. The role of complement in C3 glomerulopathy. Mol Immunol. 2015;67:21–30. [PubMed: 25929733]

Zipfel PF, Smith RJH, Heinen ST. The role of complement in membranoproliferative glomerulonephritis. In: Zipfel PF, ed. Complement and Kidney Disease. Berlin, Germany: Springer; 2006:199-221.