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

N. Garam, M. Cserhalmi, Z. Prohászka, Á. Szilágyi, N. Veszeli, E. Szabó, B. Uzonyi, A. Iliás, C. Aigner, A. Schmidt, M. Gaggl, G. Sunder-Plassmann, D. Bajcsi, J. Brunner, A. Dumfarth, D. Cejka, S. Flaschberger, H. Flögelova, Á. Haris, Á....

. 2021 ; 12 (-) : 720183. [pub] 20210910

Jazyk angličtina Země Švýcarsko

Typ dokumentu časopisecké články, práce podpořená grantem

Perzistentní odkaz   https://www.medvik.cz/link/bmc22003743

Background: 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. Methods: 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). Results: 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. Conclusions: Our observations raise the possibility that the FHR-5 protein plays a fine-tuning role in the pathogenesis of IC-MPGN/C3G.

1st Department of Internal Medicine University of Szeged Szeged Hungary

1st Department of Pediatrics Semmelweis University Budapest Hungary

Carol Davila Nephrology Hospital Bucharest Romania

Department of Immunology ELTE Eötvös Loránd University Budapest Hungary

Department of Internal Medicine 4 Nephrology and Hypertension Medical University Innsbruck Innsbruck Austria

Department of Internal Medicine and Haematology Semmelweis University Budapest Hungary

Department of Internal Medicine and Oncology Semmelweis University Budapest Hungary

Department of Medicine 3 Nephrology Transplant Medicine and Rheumatology Geriatric Department Ordensklinikum Linz Elisabethinen Linz Austria

Department of Nephrology Arterial Hypertension Dialysis and Transplantation University Hospital Center Zagreb School of Medicine University of Zagreb Zagreb Croatia

Department of Nephrology Hypertension and Internal Medicine School of Medicine Collegium Medicum University of Warmia and Mazury Olsztyn Poland

Department of Nephrology Péterfy Hospital Budapest Hungary

Department of Nephrology University Hospital Dubrava Zagreb Zagreb Croatia

Department of Nephrology Wilhelminenspital Vienna Austria

Department of Pathology Tartu University Hospital Tartu Estonia

Department of Pediatric Nephrology Division of Pediatrics University Medical Center Ljubljana Ljubljana Slovenia

Department of Pediatrics 2nd Faculty of Medicine Charles University Prague University Hospital Motol Pragu Czechia

Department of Pediatrics and Adolescent Medicine Division of Pediatric Nephrology and Gastroenterology Medical University of Vienna Vienna Austria

Department of Pediatrics Comenius University Bratislava Slovakia

Department of Pediatrics Faculty of Medicine Debrecen University Debrecen Hungary

Department of Pediatrics Faculty of Medicine in Pilsen Charles University Prague Pilsen Czechia

Department of Pediatrics Medical University of Innsbruck Innsbruck Austria

Department of Pediatrics University Hospital and Faculty of Medicine Ostrava Czechia

Department of Pediatrics University Hospital Split Split Croatia

Department of Pediatrics University of Pécs Pécs Hungary

Department of Pediatrics University of Szeged Szeged Hungary

Division of Nephrology and Dialysis Department of Medicine 3 Medical University of Vienna Vienna Austria

Division of Nephrology Department of Pediatrics Faculty of Medicine Palacky University and Faculty Hospital in Olomouc Olomouc Czechia

Fresenius Medical Care Center of Dialysis Miskolc Hungary

Hospital of Klagenfurt Klagenfurt Austria

Institute of Mother and Childhealth Care of Serbia Dr Vukan Čupić Belgrade Serbia

Institute of Neurology of Senses and Language Hospital of St John of God Linz Austria

Institute of Pathology Faculty of Medicine University of Ljubljana Ljubljana Slovenia

Internal Medicine 4 Section of Nephrology Klinikum Wels Grieskirchen Wels Austria

Medimpax Bratislava Slovakia

MTA ELTE Complement Research Group Eötvös Loránd Research Network Department of Immunology ELTE Eötvös Loránd University Budapest Hungary

Nephrology Center Santaros Klinikos Medical Faculty Vilnius University Vilnius Lithuania

Nephrology Clinic 1st Faculty of Medicine Charles University Prague Czechia

Pediatric Nephrology Department Fundeni Clinical Institute Bucharest Romania

Research Group for Immunology and Haematology Semmelweis University Eötvös Loránd Research Network Budapest Hungary

Research Institute for Developmental Medicine Johannes Kepler University Linz Linz Austria

School of Medicine University of Split Split Croatia

University Children's Hospital Medical University Sofia Bulgaria

Citace poskytuje Crossref.org

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$a Background: Factor H-related protein 5 (FHR-5) is a member of the complement Factor H protein family. Due to the homology to Factor H, the main complement regulator of the alternative pathway, it may also be implicated in the pathomechanism of kidney diseases where Factor H and alternative pathway dysregulation play a role. Here, we report the first observational study on CFHR5 variations along with serum FHR-5 levels in immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G) patients together with the clinical, genetic, complement, and follow-up data. Methods: A total of 120 patients with a histologically proven diagnosis of IC-MPGN/C3G were enrolled in the study. FHR-5 serum levels were measured in ELISA, the CFHR5 gene was analyzed by Sanger sequencing, and selected variants were studied as recombinant proteins in ELISA and surface plasmon resonance (SPR). Results: Eight exonic CFHR5 variations in 14 patients (12.6%) were observed. Serum FHR-5 levels were lower in patients compared to controls. Low serum FHR-5 concentration at presentation associated with better renal survival during the follow-up period; furthermore, it showed clear association with signs of complement overactivation and clinically meaningful clusters. Conclusions: Our observations raise the possibility that the FHR-5 protein plays a fine-tuning role in the pathogenesis of IC-MPGN/C3G.
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$a Jakab, Dániel $u Department of Pediatrics, University of Szeged, Szeged, Hungary
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$a Szigeti, Erika $u Department of Pediatrics, University of Szeged, Szeged, Hungary
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$a Bereczki, Csaba $u Department of Pediatrics, University of Szeged, Szeged, Hungary
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$a Szabó, Attila J $u 1st Department of Pediatrics, Semmelweis University, Budapest, Hungary
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$a Zlatanova, Galia $u University Children's Hospital, Medical University, Sofia, Bulgaria
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