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

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

Factor 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...

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Main Authors: Garam Nóra
Cserhalmi Marcell
Prohászka Zoltán
Szilágyi Ágnes
Veszeli Nóra
Szabó Edina Erzsébet
Uzonyi Barbara
Iliás Attila
Bajcsi Dóra
Haris Ágnes
Jakab Dániel
Sinkó Mária
Szigeti Erika
Bereczki Csaba
Kelen Kata
Reusz György
Szabó Attila
Szabó Tamás
Tislér András
Józsi Mihály
Csuka Dorottya
Format: Article
Published: 2021
Series:FRONTIERS IN IMMUNOLOGY 12
Subjects:
Klinikai orvostan
doi:10.3389/fimmu.2021.720183

mtmt:32266555
Online Access:http://publicatio.bibl.u-szeged.hu/26768
Description
Summary:Factor 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.A 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).Eight 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.Our observations raise the possibility that the FHR-5 protein plays a fine-tuning role in the pathogenesis of IC-MPGN/C3G.
Physical Description:20
ISSN:1664-3224

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