Zhang Y, Martin B, Spies MA, Roberts SM, Nott J, Goodfellow RX, Nelson AFM, Blain SJ, Redondo E, Nester CM, Smith RJH. Renin and renin blockade have no role in complement activity. Kidney Int. 2024 Feb;105(2):328-337. doi: 10.1016/j.kint.2023.11.005. Epub 2023 Nov 25. PMID: 38008161; PMCID: PMC10872535.

Donelson CJH, Ghiringhelli Borsa N, Taylor AO, Smith RJH, Zhang Y. Functional evaluation of rare variants in complement factor I using a minigene assay. Front Immunol. 2024 Aug 22;15:1446081. doi: 10.3389/fimmu.2024.1446081. PMID: 39238643; PMCID: PMC11374653.

Culek, Christopher T.; Nester, Carla M.. A Nef Precursor or Benign Counterpart: High Prevalence of Healthy Subjects with Antibody Reactivity to the C3-Convertase. Kidney360 4(11):p 1615-1622, November 2023. | DOI: 10.34067/KID.0000000000000260
 

Schmidt CQ, Smith RJH. Protein therapeutics and their lessons: Expect the unexpected when inhibiting the multi-protein cascade of the complement system. Immunol Rev. 2023 Jan;313(1):376-401. doi: 10.1111/imr.13164. Epub 2022 Nov 18. PMID: 36398537.

Pisarenka S, Meyer NC, Xiao X, Goodfellow R, Nester CM, Zhang Y, Smith RJH. Modeling C3 glomerulopathies: C3 convertase regulation on an extracellular matrix surface. Front Immunol. 2023 Jan 18;13:1073802. doi: 10.3389/fimmu.2022.1073802. PMCID: PMC9947773.

Marina Vivarelli, Andrew S. Bomback, Matthias Meier, Yaqin Wang, Nicholas J.A. Webb, Uday Kiran Veldandi, Richard J.H. Smith, David Kavanagh, Iptacopan in Idiopathic Immune Complex–Mediated Membranoproliferative Glomerulonephritis: Protocol of the APPARENT Multicenter, Randomized Phase 3 Study, Kidney International Reports, 2023, ISSN 2468-0249, (https://www.sciencedirect.com/science/article/pii/S2468024923015620)
 

U.K. VELDANDI, D. Kavanagh, M. Vivarelli, A. Bomback, Y. Wang, K. Bogdanowicz, N. Webb, M. Meier, R.J.H. Smith,
WCN23-0584 A MULTICENTER, RANDOMIZED, DOUBLE-BLIND, PLACEBO-CONTROLLED PHASE 3 STUDY TO ASSESS THE EFFICACY AND SAFETY OF IPTACOPAN IN IDIOPATHIC IMMUNE COMPLEX-MEDIATED MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS (IC-MPGN) Kidney International Reports, Volume 8, Issue 3, Supplement, 2023, Page S275, ISSN 2468-0249, 
 

Michael M, Bagga A, Sartain SE, Smith RJH. Haemolytic uraemic syndrome. Lancet. 2022 Nov 12;400(10364):1722-1740. doi: 10.1016/S0140-6736(22)01202-8. Epub 2022 Oct 19. PMID: 36272423.

Bomback AS, Kavanagh D, Vivarelli M, Meier M, Wang Y, Webb NJA, Trapani AJ, Smith RJH. Alternative Complement Pathway Inhibition With Iptacopan for the Treatment of C3 Glomerulopathy-Study Design of the APPEAR-C3G Trial. Kidney Int Rep. 2022 Aug 2;7(10):2150-2159. doi: 10.1016/j.ekir.2022.07.004. PMID: 36217526.

Heiderscheit AK, Hauer JJ, Smith RJH. C3 glomerulopathy: Understanding an ultra-rare complement-mediated renal disease. Am J Med Genet C Semin Med Genet. 2022 Jun 23. doi: 10.1002/ajmg.c.31986. Epub ahead of print. PMID: 35734939.

Chauvet S, Hauer JJ, Petitprez F, Rabant M, Martins PV, Baudouin V, Delmas Y, Jourde-Chiche N, Cez A, Ribes D, Cloarec S, Servais A, Zaidan M, Daugas E, Delahousse M, Wynckel A, Ryckewaert A, Sellier-Leclerc AL, Boyer O, Thervet E, Karras A, Smith RJH, Frémeaux-Bacchi V. Results from a national-wide retrospective cohort measure the impact of C3 and soluble C5b-9 levels on kidney outcomes in C3 glomerulopathy. Kidney Int. 2022 Jun 22:S0085-2538(22)00464-1. doi: 10.1016/j.kint.2022.05.027. Epub ahead of print. PMID: 35752323.

Zhang Y, Goodfellow RX, Ghiringhelli Borsa N, Dunlop HC, Presti SA, Meyer NC, Shao D, Roberts SM, Jones MB, Pitcher GR, Taylor AO, Nester CM, Smith RJH. Complement Factor I Variants in Complement-Mediated Renal Diseases. Front Immunol. 2022 May 10;13:866330. doi: 10.3389/fimmu.2022.866330. PMID: 35619721.

Rodriguez KM, Vaught J, Dilley M, Ellsworth K, Heinen A, Abud EM, Zhang Y, Smith RJH, Sheets R, Geng B, Hoffman HM, Worthen HM, Dimmock D, Coufal NG. Rapid genome sequencing identifies novel variants in complement factor I. Cold Spring Harb Mol Case Stud. 2022 Dec 28;8(7):a006239. doi: 10.1101/mcs.a006239. PMID: 36577522; PMCID: PMC9808552.

Chauvet S, Hauer JJ, Petitprez F, Rabant M, Martins PV, Baudouin V, Delmas Y, Jourde-Chiche N, Cez A, Ribes D, Cloarec S, Servais A, Zaidan M, Daugas E, Delahousse M, Wynckel A, Ryckewaert A, Sellier-Leclerc AL, Boyer O, Thervet E, Karras A, Smith RJH, Frémeaux-Bacchi V. Results from a nationwide retrospective cohort measure the impact of C3 and soluble C5b-9 levels on kidney outcomes in C3 glomerulopathy. Kidney Int. 2022 Oct;102(4):904-916. doi: 10.1016/j.kint.2022.05.027. Epub 2022 Jun 22. PMID: 35752323; PMCID: PMC10588728.

J. HAUER, N. Meyer, R. Goodfellow, C. Nester, Y. Zhang, R. Smith
POS-017 A POPULATION PERSPECTIVE OF NEPHRITIC FACTORS AS SYSTEMIC DRIVERS OF COMPLEMENT DYSREGULATION IN C3 GLOMERULOPATHY,
Kidney International Reports, Volume 7, Issue 6, Supplement, 2022, Page S444,
ISSN 2468-0249, https://doi.org/10.1016/j.ekir.2022.04.036.

Y. ZHANG, N. Ghiringhelli Borsa, R. Goodfellow, H. Dunlop, N. Meyer, D. Shao, M. Jones, G. Pitcher, A. Taylor, C. Nester, R. Smith
POS-024 COMPLEMENT FACTOR I VARIANTS IN ATYPICAL HEMOLYTIC UREMIC SYNDROME AND C3 GLOMERULOPATHY,
Kidney International Reports, Volume 7, Issue 6, Supplement, 2022, Page S448,
ISSN 2468-0249, https://doi.org/10.1016/j.ekir.2022.04.044.
(https://www.sciencedirect.com/science/article/pii/S2468024922013213)
 

Zhang Y, Goodfellow RX, Ghiringhelli Borsa N, Dunlop HC, Presti SA, Meyer NC, Shao D, Roberts SM, Jones MB, Pitcher GR, Taylor AO, Nester CM, Smith RJH. Complement Factor I Variants in Complement-Mediated Renal Diseases. Front Immunol. 2022 May 10;13:866330. doi: 10.3389/fimmu.2022.866330. PMID: 35619721; PMCID: PMC9127439.

Bomback AS, Appel GB, Gipson DS, Hladunewich MA, Lafayette R, Nester CM, Parikh SV, Smith RJH, Trachtman H, Heeger PS, Ram S, Rovin BH, Ali S, Arceneaux N, Ashoor I, Bailey-Wickins L, Barratt J, Beck L, Cattran DC, Cravedi P, Erkan E, Fervenza F, Frazer-Abel AA, Fremeaux-Bacchi V, Fuller L, Gbadegesin R, Hogan JJ, Kiryluk K, le Quintrec-Donnette M, Licht C, Mahan JD, Pickering MC, Quigg R, Rheault M, Ronco P, Sarwal MM, Sethna C, Spino C, Stegall M, Vivarelli M, Feldman DL, Thurman JM. Improving Clinical Trials for Anticomplement Therapies in Complement-Mediated Glomerulopathies: Report of a Scientific Workshop Sponsored by the National Kidney Foundation. Am J Kidney Dis. 2022 Apr;79(4):570-581. doi: 10.1053/j.ajkd.2021.07.025. Epub 2021 Sep 24. PMID: 34571062.

Loeven MA, Maciej-Hulme ML, Yanginlar C, Hubers MC, Kellenbach E, de Graaf M, van Kuppevelt TH, Wetzels J, Rabelink TJ, Smith RJH, van der Vlag J. Selective Binding of Heparin/Heparan Sulfate Oligosaccharides to Factor H and Factor H-Related Proteins: Therapeutic Potential for C3 Glomerulopathies. Front Immunol. 2021 Aug 18;12:676662. doi: 10.3389/fimmu.2021.676662. PMID: 34489931.

Martin Merinero H, Zhang Y, Arjona E, Del Angel G, Goodfellow R, Gomez-Rubio E, Ji RR, Michelena M, Smith RJH, Rodríguez de Córdoba S. Functional characterization of 105 Factor H variants associated with atypical HUS: lessons for variant classification. Blood. 2021 Jun 29:blood.2021012037. doi: 10.1182/blood.2021012037. Epub ahead of print. PMID: 34189567.

Johnson CK, Zuniga SC, Dhawale T, Zhang Y, Smith RJH, Blosser CD. Monoclonal Gammopathy of Renal Significance Causes C3 Glomerulonephritis Via Monoclonal IgG Kappa Inhibition of Complement Factor H. Kidney Int Rep. 2021 Jun 27;6(9):2505-2509. doi: 10.1016/j.ekir.2021.06.015. PMID: 34514215.

Piras R, Breno M, Valoti E, Alberti M, Iatropoulos P, Mele C, Bresin E, Donadelli R, Cuccarolo P, Smith RJH, Benigni A, Remuzzi G, Noris M. CFH and CFHR Copy Number Variations in C3 Glomerulopathy and Immune Complex-Mediated Membranoproliferative Glomerulonephritis. Front Genet. 2021 Jun 11;12:670727. doi: 10.3389/fgene.2021.670727. PMID: 34211499; PMCID: PMC8240960.

Mansilla MA, Sompallae RR, Nishimura CJ, Kwitek AE, Kimble MJ, Freese ME, Campbell CA, Smith RJ, Thomas CP. Targeted broad-based genetic testing by next-generation sequencing informs diagnosis and facilitates management in patients with kidney diseases. Nephrol Dial Transplant. 2021 Jan 25;36(2):295-305. doi: 10.1093/ndt/gfz173. PMID: 31738409; PMCID: PMC7834596.

Rovin BH, Adler SG, Barratt J, Bridoux F, Burdge KA, Chan TM, Cook HT, Fervenza FC, Gibson KL, Glassock RJ, Jayne DRW, Jha V, Liew A, Liu ZH, Mejía-Vilet JM, Nester CM, Radhakrishnan J, Rave EM, Reich HN, Ronco P, Sanders JF, Sethi S, Suzuki Y, Tang SCW, Tesar V, Vivarelli M, Wetzels JFM, Lytvyn L, Craig JC, Tunnicliffe DJ, Howell M, Tonelli MA, Cheung M, Earley A, Floege J. Executive summary of the KDIGO 2021 Guideline for the Management of Glomerular Diseases. Kidney Int. 2021 Oct;100(4):753-779. doi: 10.1016/j.kint.2021.05.015. PMID: 34556300.

Nester C, Appel GB, Bomback AS, Bouman KP, Cook HT, Daina E, Dixon BP, Rice K, Najafian N, Hui J, Podos SD, Langman CB, Lightstone L, Parikh SV, Pickering MC, Sperati CJ, Trachtman H, Tumlin J, de Vries AP, Wetzels JFM, Remuzzi G. Clinical Outcomes of Patients with C3G or IC-MPGN Treated with the Factor D Inhibitor Danicopan: Final Results from Two Phase 2 Studies. Am J Nephrol. 2022;53(10):687-700. doi: 10.1159/000527167. Epub 2022 Nov 24. Erratum in: Am J Nephrol. 2022;53(11-12):859. doi: 10.1159/000528430. PMID: 36423588.

Edwin Kwan Soon Wong, Manuel Praga, Carla Nester, Moglie Le Quintrec, Erica Daina, Giuseppe Remuzzi, Angelo Trapani, Yaqin Wang, Junhao Liu, Guido Junge, Matthias Meier, Nicholas Webb, FC 036
IPTACOPAN (LNP023): A NOVEL ORAL COMPLEMENT ALTERNATIVE PATHWAY FACTOR B INHIBITOR SAFELY AND EFFECTIVELY STABILISES EGFR IN C3 GLOMERULOPATHY, Nephrology Dialysis Transplantation, Volume 36, Issue Supplement_1, May 2021, gfab121.005, https://doi.org/10.1093/ndt/gfab121.005

Carla Nester, Patrick Breheny, Monica Hall, Alan Charney, Martin Lefkowitz, Angelo Trapani, Yaqin Wang, Richard Smith, MO136
RELATIONSHIP BETWEEN UPCR AND EGFR IN C3 GLOMERULOPATHY, Nephrology Dialysis Transplantation, Volume 36, Issue Supplement_1, May 2021

Zhang Y, Ghiringhelli Borsa N, Shao D, Dopler A, Jones MB, Meyer NC, Pitcher GR, Taylor AO, Nester CM, Schmidt CQ, Smith RJH. Factor H Autoantibodies and Complement-Mediated Diseases. Front Immunol. 2020 Dec 15;11:607211. doi: 10.3389/fimmu.2020.607211. PMID: 33384694.

Zhang Y, Kremsdorf R, Sperati CJ, et al. Mutation of complement factor B causing massive fluid-phase dysregulation of the alternative complement pathway can result in atypical hemolytic uremic syndrome [published online ahead of print, 2020 Jun 12]. Kidney Int. 2020;S0085-2538(20)30637-2. doi:10.1016/j.kint.2020.05.028. PMID: 32540405.

Zhang Y, Keenan A, Dai DF, May KS, Anderson EE, Lindorfer MA, Henrich JB, Pitcher GR, Taylor RP, Smith RJ. C3(H2O) prevents rescue of complement-mediated C3 glomerulopathy in Cfh-/- Cfd-/- mice. JCI Insight. 2020 May 7;5(9):e135758. doi: 10.1172/jci.insight.135758. PMID: 32376801.

Schubart A, Anderson K, Mainolfi N, Sellner H, Ehara T, Adams CM, Mac Sweeney A, Liao SM, Crowley M, Littlewood-Evans A, Sarret S, Wieczorek G, Perrot L, Dubost V, Flandre T, Zhang Y, Smith RJH, Risitano AM, Karki RG, Zhang C, Valeur E, Sirockin F, Gerhartz B, Erbel P, Hughes N, Smith TM, Cumin F, Argikar UA, Haraldsson B, Mogi M, Sedrani R, Wiesmann C, Jaffee B, Maibaum J, Flohr S, Harrison R, Eder J. Small-molecule factor B inhibitor for the treatment of complement-mediated diseases. Proc Natl Acad Sci U S A. 2019 Apr 16;116(16):7926-7931. doi: 10.1073/pnas.1820892116. Epub 2019 Mar 29. PMID: 30926668.

Hauer JJ, Shao D, Zhang Y, Nester CM, Smith RJH. Factor B and C4b2a Autoantibodies in C3 Glomerulopathy. Front Immunol. 2019 Apr 4;10:668. doi:10.3389/fimmu.2019.00668. eCollection 2019. Review. PMID: 31024533.

Smith RJH, Appel GB, Blom AM, Cook HT, D'Agati VD, Fakhouri F, Fremeaux-Bacchi V, Józsi M, Kavanagh D, Lambris JD, Noris M, Pickering MC, Remuzzi G, de Córdoba  SR, Sethi S, Van der Vlag J, Zipfel PF, Nester CM. C3 glomerulopathy - understanding a rare complement-driven renal disease. Nat Rev Nephrol. 2019 Mar;15(3):129-143. doi: 10.1038/s41581-018-0107-2. Review. PMID: 30692664.

Aishwarya Ravindran, Benjamin Madden, M. Cristine Charlesworth, Rishi Sharma, Amit Sethi, Hanna Debiec, Daniel Cattran, Fernando C. Fervenza, Richard J. Smith, Pierre Ronco, Sanjeev Sethi
Proteomic Analysis of Complement Proteins in Membranous Nephropathy, Kidney International Reports, Volume 5, Issue 5, 2020, Pages 618-626, ISSN 2468-0249, https://doi.org/10.1016/j.ekir.2020.01.018.
(https://www.sciencedirect.com/science/article/pii/S2468024920300395)

Andrea L. Oliverio, Jarcy Zee, Laura H. Mariani, Monica L. Reynolds, Michelle O’Shaughnessy, Elizabeth M. Hendren, Nada Alachkar, Emily Herreshoff, Dana V. Rizk, Carla M. Nester, Julia Steinke, Katherine E. Twombley, Michelle A. Hladunewich,
Renal Complications in Pregnancy Preceding Glomerulonephropathy Diagnosis,
Kidney International Reports, Volume 4, Issue 1, 2019, Pages 159-162, ISSN 2468-0249, https://doi.org/10.1016/j.ekir.2018.10.012.
(https://www.sciencedirect.com/science/article/pii/S2468024918302833)
 

Shin HS, Nester CM, Dixon BP. Comorbidity of inflammatory bowel disease with atypical hemolytic uremic syndrome in pediatric patients. Clin Nephrol Case Stud. 2019 Jun 25;7:35-40. doi: 10.5414/CNCS109511. PMID: 31312592; PMCID: PMC6595396.