C3 glomerulopathy—understanding a rare complement-driven renal disease

RJH Smith, GB Appel, AM Blom, HT Cook… - Nature reviews …, 2019 - nature.com
The C3 glomerulopathies are a group of rare kidney diseases characterized by complement
dysregulation occurring in the fluid phase and in the glomerular microenvironment, which …

Exploring the genetic basis of early-onset chronic kidney disease

A Vivante, F Hildebrandt - Nature Reviews Nephrology, 2016 - nature.com
The primary causes of chronic kidney disease (CKD) in children differ from those of CKD in
adults. In the USA the most common diagnostic groups of renal disease that manifest before …

[HTML][HTML] Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes”(KDIGO) Controversies …

THJ Goodship, HT Cook, F Fakhouri, FC Fervenza… - Kidney international, 2017 - Elsevier
In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G)
complement plays a primary role in disease pathogenesis. Herein we report the outcome of …

[HTML][HTML] C3 glomerulopathy: consensus report

MC Pickering, VD D'agati, CM Nester, RJ Smith… - Kidney international, 2013 - Elsevier
C3 glomerulopathy is a recently introduced pathological entity whose original definition was
glomerular pathology characterized by C3 accumulation with absent or scanty …

Membranoproliferative glomerulonephritis—a new look at an old entity

S Sethi, FC Fervenza - New England Journal of Medicine, 2012 - Mass Medical Soc
This review discusses the causes, pathogenesis, and clinical management of
membranoproliferative glomerulonephritis, which accounts for 7 to 10% of biopsy-confirmed …

C3 glomerulopathy: clinicopathologic features and predictors of outcome

NR Medjeral-Thomas… - Clinical Journal of the …, 2014 - journals.lww.com
Results Eighty patients with C3 glomerulopathy were identified: 21 with DDD and 59 with
C3GN. Patients with DDD were younger, more likely to have low serum C3 levels, and more …

[HTML][HTML] Complement factor H related proteins (CFHRs)

C Skerka, Q Chen, V Fremeaux-Bacchi… - Molecular …, 2013 - Elsevier
Factor H related proteins comprise a group of five plasma proteins: CFHR1, CFHR2,
CFHR3, CFHR4 and CFHR5, and each member of this group binds to the central …

Statistical validation of rare complement variants provides insights into the molecular basis of atypical hemolytic uremic syndrome and C3 glomerulopathy

AJ Osborne, M Breno, NG Borsa, F Bu… - The Journal of …, 2018 - journals.aai.org
Atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) are associated
with dysregulation and overactivation of the complement alternative pathway. Typically …

[HTML][HTML] Effectiveness of mycophenolate mofetil in C3 glomerulonephritis

C Rabasco, T Cavero, E Román, J Rojas-Rivera… - Kidney international, 2015 - Elsevier
C3 glomerulonephritis is a clinicopathologic entity defined by the presence of isolated or
dominant deposits of C3 on immunofluorescence. To explore the effect of …

[HTML][HTML] Toward a working definition of C3 glomerulopathy by immunofluorescence

J Hou, GS Markowitz, AS Bomback, GB Appel… - Kidney international, 2014 - Elsevier
Precise immunofluorescence criteria for C3 glomerulopathy remain to be defined. Here we
tested hierarchical immunofluorescence criteria with varying stringency for C3 …