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FHR-5 Serum Levels and CFHR5 Genetic Variations in Patients With Immune Complex-Mediated Membranoproliferative Glomerulonephritis and C3-Glomerulopathy

Authors :
Nóra Garam
Marcell Cserhalmi
Zoltán Prohászka
Ágnes Szilágyi
Nóra Veszeli
Edina Szabó
Barbara Uzonyi
Attila Iliás
Christof Aigner
Alice Schmidt
Martina Gaggl
Gere Sunder-Plassmann
Dóra Bajcsi
Jürgen Brunner
Alexandra Dumfarth
Daniel Cejka
Stefan Flaschberger
Hana Flögelova
Ágnes Haris
Ágnes Hartmann
Andreas Heilos
Thomas Mueller
Krisztina Rusai
Klaus Arbeiter
Johannes Hofer
Dániel Jakab
Mária Sinkó
Erika Szigeti
Csaba Bereczki
Viktor Janko
Kata Kelen
György S. Reusz
Attila J. Szabó
Nóra Klenk
Krisztina Kóbor
Nika Kojc
Maarten Knechtelsdorfer
Mario Laganovic
Adrian Catalin Lungu
Anamarija Meglic
Rina Rus
Tanja Kersnik Levart
Ernesta Macioniene
Marius Miglinas
Anna Pawłowska
Tomasz Stompór
Ludmila Podracka
Michael Rudnicki
Gert Mayer
Romana Rysava
Jana Reiterova
Marijan Saraga
Tomáš Seeman
Jakub Zieg
Eva Sládková
Natasa Stajic
Tamás Szabó
Andrei Capitanescu
Simona Stancu
Miroslav Tisljar
Kresimir Galesic
András Tislér
Inga Vainumäe
Martin Windpessl
Tomas Zaoral
Galia Zlatanova
Mihály Józsi
Dorottya Csuka
Source :
Frontiers in immunology, Lausanne : Frontiers Media S.A., 2021, vol. 12, art. no. 720183, p. [1-20], Frontiers in Immunology, Vol 12 (2021), Frontiers in Immunology
Publication Year :
2021

Abstract

BackgroundFactor H-related protein 5 (FHR-5) is a member of the complement Factor H protein family. Due to the homology to Factor H, the main complement regulator of the alternative pathway, it may also be implicated in the pathomechanism of kidney diseases where Factor H and alternative pathway dysregulation play a role. Here, we report the first observational study on CFHR5 variations along with serum FHR-5 levels in immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G) patients together with the clinical, genetic, complement, and follow-up data.MethodsA total of 120 patients with a histologically proven diagnosis of IC-MPGN/C3G were enrolled in the study. FHR-5 serum levels were measured in ELISA, the CFHR5 gene was analyzed by Sanger sequencing, and selected variants were studied as recombinant proteins in ELISA and surface plasmon resonance (SPR).ResultsEight exonic CFHR5 variations in 14 patients (12.6%) were observed. Serum FHR-5 levels were lower in patients compared to controls. Low serum FHR-5 concentration at presentation associated with better renal survival during the follow-up period; furthermore, it showed clear association with signs of complement overactivation and clinically meaningful clusters.ConclusionsOur observations raise the possibility that the FHR-5 protein plays a fine-tuning role in the pathogenesis of IC-MPGN/C3G.

Details

Language :
English
ISSN :
16643224
Database :
OpenAIRE
Journal :
Frontiers in immunology, Lausanne : Frontiers Media S.A., 2021, vol. 12, art. no. 720183, p. [1-20], Frontiers in Immunology, Vol 12 (2021), Frontiers in Immunology
Accession number :
edsair.doi.dedup.....6159a9f548a35b3f0dbb1659bdbdfd7c