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FHR-5 Serum Levels and CFHR5 Genetic Variations in Patients With Immune Complex-Mediated Membranoproliferative Glomerulonephritis and C3-Glomerulopathy
- 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.
- Subjects :
- Male
membranoproliferative glomerulonephritis
immune complex-mediated glomerulonephritis
Antigen-Antibody Complex
Kidney Function Tests
Pathogenesis
Membranoproliferative glomerulonephritis
Medicine
Immunology and Allergy
03.02. Klinikai orvostan
Complement Activation
Original Research
Disease Management
Complement C3
Prognosis
Immune complex
C3 glomerulonephritis (C3GN)
C3 glomerulopathy
dense deposit disease (DDD)
Factor H
embryonic structures
Female
Disease Susceptibility
Symptom Assessment
Adult
Adolescent
Glomerulonephritis, Membranoproliferative
Immunology
Enzyme-Linked Immunosorbent Assay
Polymorphism, Single Nucleotide
Young Adult
Immune system
Glomerulopathy
Humans
Genetic Predisposition to Disease
Alleles
business.industry
Genetic Variation
Complement System Proteins
RC581-607
medicine.disease
ROC Curve
Case-Control Studies
Alternative complement pathway
Immunologic diseases. Allergy
business
CFHR5
Biomarkers
Subjects
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