Nephritic factors: an overview of classification, diagnostic tools and clinical associations

F Corvillo, M Okrój, P Nozal, M Melgosa… - Frontiers in …, 2019 - frontiersin.org
Nephritic factors comprise a heterogeneous group of autoantibodies against neoepitopes
generated in the C3 and C5 convertases of the complement system, causing its …

Testing the activity of complement convertases in serum/plasma for diagnosis of C4NeF-mediated C3 glomerulonephritis

AM Blom, F Corvillo, M Magda, G Stasiłojć… - Journal of clinical …, 2016 - Springer
Autoantibodies termed C3-nephritic factor (C3NeF), which stabilize convertases of the
alternative complement pathway, often stimulate autoinflammatory diseases. However …

Autoantibody stabilization of the classical pathway C3 convertase leading to C3 deficiency and Neisserial sepsis: C4 nephritic factor revisited

EC Miller, NM Chase, P Densen, MK Hintermeyer… - Clinical …, 2012 - Elsevier
C3 deficiency is a rare disorder that leads to recurrent pyogenic infections. Here we describe
a previously healthy 18 y/o Caucasian male with severe meningococcal disease. Total …

Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome

M Józsi, S Reuter, P Nozal, M López-Trascasa… - Immunology letters, 2014 - Elsevier
The alternative pathway of complement is implicated in the pathogenesis of several renal
diseases, such as atypical hemolytic uremic syndrome, dense deposit disease and other …

[HTML][HTML] C5 nephritic factors drive the biological phenotype of C3 glomerulopathies

MC Marinozzi, S Chauvet, M Le Quintrec, M Mignotet… - Kidney international, 2017 - Elsevier
C3 Glomerulopathies, which include Dense Deposit Disease and C3 Glomerulonephritis,
are associated with genetic and acquired dysregulation of the C3 convertase alternative …

[HTML][HTML] Sensitive and specific assays for C3 nephritic factors clarify mechanisms underlying complement dysregulation

D Paixao-Cavalcante, M Lopez-Trascasa, L Skattum… - Kidney international, 2012 - Elsevier
C3 nephritic factors are autoantibodies that prolong the half-life or prevent regulation of the
alternative pathway C3 convertase, resulting in uncontrolled complement activation. They …

Autoimmune abnormalities of the alternative complement pathway in membranoproliferative glomerulonephritis and C3 glomerulopathy

M Noris, R Donadelli, G Remuzzi - Pediatric Nephrology, 2019 - Springer
Membranoproliferative glomerulonephritis (MPGN) is a rare chronic kidney disease
associated with complement activation. Recent immunofluorescence-based classification …

[HTML][HTML] C4 nephritic factors in C3 glomerulopathy: a case series

Y Zhang, NC Meyer, FC Fervenza, W Lau… - American Journal of …, 2017 - Elsevier
Background C3 glomerulopathy (C3G) defines a group of rare complement-mediated kidney
diseases with a shared underlying pathophysiology: dysregulation of complement in the …

Unraveling the molecular mechanisms underlying complement dysregulation by nephritic factors in C3G and IC-MPGN

R Donadelli, P Pulieri, R Piras, P Iatropoulos… - Frontiers in …, 2018 - frontiersin.org
Membranoproliferative glomerulonephritis (MPGN) was recently classified as C3
glomerulopathies (C3G), and immune-complex (IC) mediated MPGN. Dysregulation of the …

The role of complement in C3 glomerulopathy

PF Zipfel, C Skerka, Q Chen, T Wiech, T Goodship… - Molecular …, 2015 - Elsevier
C3 glomerulopathy describes a spectrum of disorders with glomerular pathology associated
with C3 cleavage product deposition and with defective complement action and regulation …