C3 glomerulopathy: the genetic and clinical findings in dense deposit disease and C3 glomerulonephritis

X Xiao, MC Pickering, RJH Smith - Seminars in thrombosis and …, 2014 - thieme-connect.com
C3 glomerulopathy (C3G) defines a group of very rare renal diseases in which dysregulation
of the alternative and terminal complement pathways plays a pivotal pathogenic role …

C3 Glomerulopathy

A Servais, LH Noël, V Frémeaux-Bacchi… - New Insights into …, 2013 - karger.com
C3 glomerulopathy is a recent disease classification comprising several rare types of
glomerulonephritis, including dense deposit disease (DDD) and C3 glomerulonephritis …

Complement and the kidney in the setting of Shiga-toxin hemolytic uremic syndrome, organ transplantation, and C3 glomerulonephritis

LS Keir, CB Langman - Transfusion and Apheresis Science, 2016 - Elsevier
Objectives To review the role of complement in glomerular pathologies focusing on
thrombotic microangiopathies (TMA) caused by Shiga toxin (Stx) and organ transplantation …

C3-glomerulopathy and MGUS: the skin beyond the kidney

GA Ramirez, G De Luca, G Dell'Antonio… - … Journal of Medicine, 2018 - academic.oup.com
The diagnosis of C3G is often challenging, as it can mimic other inflammatory diseases.
Limited data on the pathogenesis of the disease further complicate its clinical management …

Functional characterization of the disease-associated N-terminal complement factor H mutation W198R

M Cserhalmi, B Uzonyi, NS Merle, D Csuka… - Frontiers in …, 2017 - frontiersin.org
Dysregulation of the complement alternative pathway is involved in the pathogenesis of
several diseases, including the kidney diseases atypical hemolytic uremic syndrome (aHUS) …

C3 glomerulopathy: ten years' experience at Mayo Clinic

A Ravindran, FC Fervenza, RJH Smith… - Mayo Clinic …, 2018 - Elsevier
Objective To describe the clinicopathological features, complement abnormalities, triggers,
treatment, and outcomes of C3 glomerulopathy. Patients and Methods A total of 114 patients …

Underlying Genetics of aHUS: Which Connection with Outcome and Treatment Discontinuation?

A Spasiano, D Palazzetti, L Dimartino, F Bruno… - International Journal of …, 2023 - mdpi.com
Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by a genetic
dysregulation of the alternative complement pathway, characterized by thrombocytopenia …

The development of atypical hemolytic uremic syndrome depends on complement C5

EG de Jorge, P Macor… - Journal of the …, 2011 - journals.lww.com
Gene variants in the alternative pathway of the complement system strongly associate with
atypical hemolytic uremic syndrome (aHUS), presumably by predisposing to increased …

Complement Terminal Pathway Activation and Intrarenal Immune Response in C3 Glomerulopathy

MS Meuleman, F Petitprez, MC Pickering… - Journal of the …, 2024 - journals.lww.com
Background: C3 glomerulopathy is a rare disease resulting from an overactivation of the
complement alternative pathway. Although there is also evidence of terminal pathway …

The role of the alternative pathway of complement activation in glomerular diseases

E Łukawska, M Polcyn-Adamczak, ZI Niemir - Clinical and experimental …, 2018 - Springer
The complement system (CS) has recently been recognized as a bridge between innate and
adaptive immunity that constitutes a very complex mechanism controlling the clearance of …