[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 …

C3 glomerulonephritis and thrombotic microangiopathy of renal allograft after pulmonary infection in a male with concomitant two complement factor I gene variations …

J Wen, W Wang, F Xu, J Sun, J Chen, X Ni - BMC nephrology, 2018 - Springer
Background It has been suggested that C3 glomerulonephritis (C3GN) and atypical
hemolytic-uremic syndrome (a stereotypical phenotype of thrombotic microangiopathy), two …

C3 glomerulopathy: A new complement-based entity

A De Lorenzo, S Tallón, B Hernández-Sevillano… - Revista Clínica …, 2014 - Elsevier
C3 glomerulopathy is a new, recently described entity that has changed the perspective,
treatment and classification of a number of glomerular diseases. It encompasses 2 similar …

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 …

Genetic analysis of the complement pathway in C3 glomerulopathy

W Zhao, Y Ding, J Lu, T Zhang, D Chen… - Nephrology Dialysis …, 2018 - academic.oup.com
Background C3 glomerulopathy often presents with a membranoproliferative
glomerulonephritis (MPGN) pattern, and is principally caused by unrestricted activation of …

[HTML][HTML] C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up

S Sethi, FC Fervenza, Y Zhang, L Zand, JA Vrana… - Kidney international, 2012 - Elsevier
C3 glomerulonephritis (C3GN) is a recently described disorder that typically results from
abnormalities in the alternative pathway (AP) of complement. Here, we describe the clinical …

Regulating complement in the kidney: insights from CFHR5 nephropathy

DP Gale, MC Pickering - Disease models & mechanisms, 2011 - journals.biologists.com
Complement factor H related protein 5 (CFHR5) nephropathy is a monogenic disorder of
complement regulation that is endemic in Cyprus. The disease is characterised by …

[HTML][HTML] Complete functional characterization of disease-associated genetic variants in the complement factor H gene

HM Merinero, SP García, J García-Fernández… - Kidney international, 2018 - Elsevier
Genetic analyses in atypical hemolytic uremic syndrome (aHUS) and C3-glomerulopathy
(C3G) patients have provided an excellent understanding of the genetic component of the …

Inherited deficiency of membrane cofactor protein expression and varying manifestations of recurrent atypical hemolytic uremic syndrome in a sibling pair

L Couzi, C Contin-Bordes, F Marliot, A Sarrat… - American Journal of …, 2008 - Elsevier
Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathic disorder
that may be familial or sporadic. Complement factor H (CFH), factor I, and membrane …

[HTML][HTML] C3 Glomerulopathy: Novel Treatment Paradigms

BT Estebanez, AS Bomback - Kidney International Reports, 2023 - Elsevier
Abstract C3 glomerulopathy (C3G) is diagnosed by kidney biopsy, with immunofluorescence
showing isolated or dominant C3 staining, indicating hyperactivity of the alternative …