Factor B and C4b2a Autoantibodies in C3 Glomerulopathy

Jill J Hauer; Dingwu Shao; Yuzhou Zhang; Carla M Nester; Richard J. H Smith

doi:10.3389/fimmu.2019.00668

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Factor B and C4b2a Autoantibodies in C3 Glomerulopathy

Journal article

Open access

Peer reviewed

Factor B and C4b2a Autoantibodies in C3 Glomerulopathy

Jill J Hauer, Dingwu Shao, Yuzhou Zhang, Carla M Nester and Richard J. H Smith

Frontiers in immunology, Vol.10, pp.668-668

04/04/2019

DOI: 10.3389/fimmu.2019.00668

PMCID: PMC6460050

PMID: 31024533

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Abstract

C3 Glomerulopathy (C3G) is a renal disease mediated primarily by dysregulation of the alternative pathway of complement. Complement is the cornerstone of innate immunity. It targets infectious microbes for destruction, clears immune complexes, and apoptotic cells from the circulation, and augments the humoral response. In C3G, this process becomes dysregulated, which leads to the deposition of complement proteins—including complement component C3—in the glomerular basement membrane of the kidney. Events that trigger complement are typically environmental insults like infections. Once triggered, in patients who develop C3G, complement activity is sustained by a variety of factors, including rare or novel genetic variants in complement genes and autoantibodies that alter normal complement protein function and/or regulation. Herein, we review two such autoantibodies, one to Factor B and the other to C4b2a, the C3 convertase of the classical, and lectin pathways. These two types of autoantibodies are identified in a small fraction of C3G patients and contribute marginally to the C3G phenotype.

autoantibodies

C3 convertase

C3 glomerulopathies

C5 convertase

complement dysregulation

factor B

Immunology

Details

Title: Subtitle: Factor B and C4b2a Autoantibodies in C3 Glomerulopathy
Creators: Jill J Hauer - University of Iowa
Dingwu Shao - University of Iowa
Yuzhou Zhang - University of Iowa
Carla M Nester - University of Iowa
Richard J. H Smith - University of Iowa
Resource Type: Journal article
Publication Details: Frontiers in immunology, Vol.10, pp.668-668
DOI: 10.3389/fimmu.2019.00668
PMID: 31024533
PMCID: PMC6460050
NLM abbreviation: Front Immunol
ISSN: 1664-3224
eISSN: 1664-3224
Publisher: Frontiers Media S.A
Language: English
Date published: 04/04/2019
Academic Unit: Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Anatomy and Cell Biology; Nephrology, Dialysis and Transplantation; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Otolaryngology; Internal Medicine
Record Identifier: 9984256842102771

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