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The Role of Complement Regulation in Kidney Disease

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Guest Editor
Institute of Pathology, Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia
Interests: renal pathology; complement system; immune-mediated kidney injury; molecular mechanisms of kidney diseases; nephropathology; translational nephropathology; uropathology

Special Issue Information

Dear Colleagues,

The complement system represents a fundamental component of innate immunity and plays a pivotal role in kidney homeostasis and disease. Dysregulation of complement activation is increasingly recognized as a key pathogenic mechanism in a broad spectrum of renal disorders, including glomerulonephritides, thrombotic microangiopathies, and tubulointerstitial injuries. The identification of genetic and acquired defects in complement-regulatory proteins has not only improved our understanding of disease mechanisms but has also led to the development of novel targeted therapies that reshape modern nephrology and pathology.

This Special Issue, “The Role of Complement Regulation in Kidney Disease”, aims to bring together original research articles and comprehensive reviews that explore molecular mechanisms, biomarkers, and therapeutic strategies related to complement activation and regulation in kidney diseases. Topics of interest include the structural and functional characterization of complement components, their crosstalk with immune and inflammatory pathways, and the clinical relevance of complement inhibition in renal pathology.

We warmly invite investigators to contribute their latest findings to this Special Issue, which will advance our understanding of complement-mediated renal injury and foster translational insights into precision medicine approaches in nephrology.

This Special Issue is supervised by Professor Dr. Jasmina Marković-Lipkovski and assisted by Guest Editor Assistant Professor Dr. Maja Životić (maja.zivotic@med.bg.ac.rs, Institute of Pathology, Faculty of Medicine, University of Belgrade, 11000 Belgrade, Serbia).

Prof. Dr. Jasmina Marković Lipkovski
Guest Editor

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Keywords

  • complement system
  • kidney disease
  • glomerulonephritis
  • C3 glomerulopathy
  • membranous nephropathy
  • complement regulation
  • innate immunity
  • molecular pathology

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Published Papers (1 paper)

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Review

35 pages, 845 KB  
Review
Complement Dysregulation in Kidney Diseases: Mechanisms, Biomarkers, and Emerging Targeted Therapies
by Patryk Jesiołowski, Mateusz Krzywda, Agnieszka Furmańczyk-Zawiska and Magdalena Durlik
Int. J. Mol. Sci. 2026, 27(8), 3466; https://doi.org/10.3390/ijms27083466 - 13 Apr 2026
Viewed by 561
Abstract
The complement system is the primary defense mechanism against pathogens, acting through opsonization, the membrane attack complex, and classical, lectin, or alternative pathways. These pathways result in the production of key complement components, including C3a (complement component), C5a, and C3b, which recruit inflammatory [...] Read more.
The complement system is the primary defense mechanism against pathogens, acting through opsonization, the membrane attack complex, and classical, lectin, or alternative pathways. These pathways result in the production of key complement components, including C3a (complement component), C5a, and C3b, which recruit inflammatory cells. Complement dysregulation leads to renal disease through the overproduction of anaphylatoxins or inappropriate formation of the membrane attack complex. The levels of complement components have been shown to be useful as predictive markers in acute kidney injury, especially in conditions of alternative pathway activation, and in diseases of immune complex pathology such as lupus nephritis and IgA nephropathy. Genetic defects in complement regulatory proteins result in diseases such as C3 glomerulopathy or atypical hemolytic uremic syndrome, in which uncontrolled C3 convertase activity results in renal failure. Therapeutic interventions targeting complement components, including eculizumab or pegcetacoplan, improve patient outcomes in atypical hemolytic uremic syndrome and C3 glomerulopathy, respectively, while other interventions improve renal function in IgA nephropathy. These findings underscore the dual role of the complement system, which is not only implicated in the progression of renal diseases but also provides the potential for the development of therapeutic interventions for the treatment of various forms of nephropathy. Full article
(This article belongs to the Special Issue The Role of Complement Regulation in Kidney Disease)
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