Complement C3-targeted therapy in C3 glomerulopathy, a prototype of complement-mediated kidney diseases

MS Meuleman, A Grunenwald, S Chauvet - Seminars in Immunology, 2022 - Elsevier
Abstract C3 glomerulopathy (C3G) is a rare and complex kidney disease that primarily
affects young adults. Renal outcomes remain poor in the absence of specific treatment. C3G …

Physiology and pathology of the C3 amplification cycle: A retrospective

K Peters - Immunological Reviews, 2023 - Wiley Online Library
Summary The C3 “Tickover” hypothesis, a mechanism whereby the host maintains constant
surveillance of potential invading pathogens, targeting them for elimination through …

[HTML][HTML] Efficacy and safety of iptacopan in patients with C3 glomerulopathy

E Wong, C Nester, T Cavero, A Karras… - Kidney international …, 2023 - Elsevier
Introduction Complement 3 glomerulopathy (C3G) is a rare inflammatory kidney disease
mediated by dysregulation of the alternative complement pathway. No targeted therapy …

[HTML][HTML] Complement factor I: Regulatory nexus, driver of immunopathology, and therapeutic

TM Hallam, SJ Sharp, A Andreadi, D Kavanagh - Immunobiology, 2023 - Elsevier
Complement factor I (FI) is the nexus for classical, lectin and alternative pathway
complement regulation. FI is an 88kDa plasma protein that circulates in an inactive …

[HTML][HTML] Challenges in diagnostic testing of nephritic factors

MAHM Michels, EB Volokhina… - Frontiers in …, 2022 - frontiersin.org
Nephritic factors (NeFs) are autoantibodies promoting the activity of the central enzymes of
the complement cascade, an important first line of defense of our innate immune system …

Complement gene mutations in children with C3 glomerulopathy: do they affect the response to mycophenolate mofetil?

N Günay, İ Dursun, İ Gökçe, M Akbalık Kara… - Pediatric …, 2024 - Springer
Abstract Background C3 glomerulopathy (C3G) is a complement-mediated disease.
Although genetic studies are not required for diagnosis, they are valuable for treatment …

[HTML][HTML] Glomerular Diseases Across Lifespan: Key Differences in Diagnostic and Therapeutic Approaches

M Windpessl, B Odler, IM Bajema, D Geetha… - Seminars in …, 2023 - Elsevier
Glomerular diseases are common causes of chronic kidney disease in childhood,
adolescence, and adulthood. The epidemiology of glomerular diseases differs between …

Quantifying association of early proteinuria and eGFR changes with long-term kidney failure hazard in C3G and IC-MPGN

S Masoud, K Wong, D Pitcher, L Downward… - medRxiv, 2024 - medrxiv.org
Abstract Background C3 glomerulopathy (C3G) and immune-complex
membranoproliferative glomerulonephritis (IC-MPGN) are rare disorders that frequently …

Prognostication for C3 glomerulopathy and idiopathic immunoglobulin-associated membranoproliferative glomerulonephritis

F Caravaca-Fontán, M Praga - … Journal of the American Society of …, 2022 - journals.lww.com
The field of complement kidney diseases is one of the fields of clinical nephrology in which
major strides have been made in recent decades. C3 glomerulopathy, a paradigmatic …

Diagnostic Challenges and Emerging Pathogeneses of Selected Glomerulopathies

NK Andeen, J Hou - Pediatric and Developmental Pathology, 2024 - journals.sagepub.com
Recent progress in glomerular immune complex and complement-mediated diseases have
refined diagnostic categories and informed mechanistic understanding of disease …