C3 glomerulopathy — understanding a rare complement-driven renal disease

Patterns of Clinical Response to Eculizumab in Patients With C3 Glomerulopathy

(2018) Moglie Le Quintrec et al.   AMERICAN JOURNAL OF KIDNEY DISEASES

Mycophenolate Mofetil in Combination with Steroids for Treatment of C3 Glomerulopathy

(2018) Rupali S. Avasare et al.   Clinical Journal of the American Society of Nephrology

Statistical Validation of Rare Complement Variants Provides Insights into the Molecular Basis of Atypical Hemolytic Uremic Syndrome and C3 Glomerulopathy

(2018) Amy J. Osborne et al.   JOURNAL OF IMMUNOLOGY

C3 glomerulonephritis and dense deposit disease share a similar disease course in a large United States cohort of patients with C3 glomerulopathy

(2018) Andrew S. Bomback et al.   KIDNEY INTERNATIONAL

C3 glomerulopathy associated with monoclonal Ig is a distinct subtype

(2018) Aishwarya Ravindran et al.   KIDNEY INTERNATIONAL

C3 Glomerulopathy: Ten Years' Experience at Mayo Clinic

(2018) Aishwarya Ravindran et al.   MAYO CLINIC PROCEEDINGS

Complement biomarkers as predictors of disease outcome in C3 glomerulopathy

(2018) Jill Hauer et al.   MOLECULAR IMMUNOLOGY

Diagnostic accuracy of immunofluorescence versus immunoperoxidase staining to distinguish immune complex-mediated glomerulonephritis and C3 dominant glomerulopathy

(2017) Yassine Bouatou et al.   HISTOPATHOLOGY

Anti-Factor B and Anti-C3b Autoantibodies in C3 Glomerulopathy and Ig-Associated Membranoproliferative GN

(2017) Maria Chiara Marinozzi et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

A proposal for standardized grading of chronic changes in native kidney biopsy specimens

(2017) Sanjeev Sethi et al.   KIDNEY INTERNATIONAL

A novel CFHR1-CFHR5 hybrid leads to a familial dominant C3 glomerulopathy

(2017) Shambhuprasad K. Togarsimalemath et al.   KIDNEY INTERNATIONAL

C5 nephritic factors drive the biological phenotype of C3 glomerulopathies

(2017) Maria-Chiara Marinozzi et al.   KIDNEY INTERNATIONAL

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

(2017) Timothy H.J. Goodship et al.   KIDNEY INTERNATIONAL

Complement activation in pauci-immune necrotizing and crescentic glomerulonephritis: results of a proteomic analysis

(2017) Sanjeev Sethi et al.   NEPHROLOGY DIALYSIS TRANSPLANTATION

The complement factor H-related proteins

(2016) Nicholas Medjeral-Thomas et al.   IMMUNOLOGICAL REVIEWS

Properdin: a tightly regulated critical inflammatory modulator

(2016) Adam Z. Blatt et al.   IMMUNOLOGICAL REVIEWS

Complement component C3 - The “Swiss Army Knife” of innate immunity and host defense

(2016) Daniel Ricklin et al.   IMMUNOLOGICAL REVIEWS

Mutations in Complement Factor H Impair Alternative Pathway Regulation on Mouse Glomerular Endothelial Cellsin Vitro

(2016) Markus A. Loeven et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Testing the Activity of Complement Convertases in Serum/Plasma for Diagnosis of C4NeF-Mediated C3 Glomerulonephritis

(2016) Anna M. Blom et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Complement receptor 3 mediates renal protection in experimental C3 glomerulopathy

(2016) Thomas D. Barbour et al.   KIDNEY INTERNATIONAL

Familial C3 glomerulonephritis caused by a novel CFHR5-CFHR2 fusion gene

(2016) Xue Xiao et al.   MOLECULAR IMMUNOLOGY

Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome

(2016) Paraskevas Iatropoulos et al.   MOLECULAR IMMUNOLOGY

Complement inhibition in C3 glomerulopathy

(2016) Carla M. Nester et al.   SEMINARS IN IMMUNOLOGY

Glomerular Diseases Dependent on Complement Activation, Including Atypical Hemolytic Uremic Syndrome, Membranoproliferative Glomerulonephritis, and C3 Glomerulopathy: Core Curriculum 2015

(2015) Marina Noris et al.   AMERICAN JOURNAL OF KIDNEY DISEASES

Visualization of the glomerular endothelial glycocalyx by electron microscopy using cationic colloidal thorium dioxide

(2015) Jan Hegermann et al.   HISTOCHEMISTRY AND CELL BIOLOGY

Anti–Factor H Autoantibodies in C3 Glomerulopathies and in Atypical Hemolytic Uremic Syndrome: One Target, Two Diseases

(2015) Caroline Blanc et al.   JOURNAL OF IMMUNOLOGY

Factor H–Related Protein 5 Interacts with Pentraxin 3 and the Extracellular Matrix and Modulates Complement Activation

(2015) Ádám I. Csincsi et al.   JOURNAL OF IMMUNOLOGY

High-Throughput Genetic Testing for Thrombotic Microangiopathies and C3 Glomerulopathies

(2015) F. Bu et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Kidney Disease Caused by Dysregulation of the Complement Alternative Pathway: An Etiologic Approach

(2015) A. S. De Vriese et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Complement Factor H-Related 5-Hybrid Proteins Anchor Properdin and Activate Complement at Self-Surfaces

(2015) Q. Chen et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Effectiveness of mycophenolate mofetil in C3 glomerulonephritis

(2015) Cristina Rabasco et al.   KIDNEY INTERNATIONAL

Factor H-related proteins determine complement-activating surfaces

(2015) Mihály Józsi et al.   TRENDS IN IMMUNOLOGY

Global Analysis Reveals the Complexity of the Human Glomerular Extracellular Matrix

(2014) R. Lennon et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Resolution of the three dimensional structure of components of the glomerular filtration barrier

(2014) Kenton P Arkill et al.   BMC Nephrology

C3 Glomerulopathy: Clinicopathologic Features and Predictors of Outcome

(2013) N. R. Medjeral-Thomas et al.   Clinical Journal of the American Society of Nephrology

C3 glomerulopathy–associated CFHR1 mutation alters FHR oligomerization and complement regulation

(2013) Agustín Tortajada et al.   JOURNAL OF CLINICAL INVESTIGATION

Complement factor H–related hybrid protein deregulates complement in dense deposit disease

(2013) Qian Chen et al.   JOURNAL OF CLINICAL INVESTIGATION

Clinical Findings, Pathology, and Outcomes of C3GN after Kidney Transplantation

(2013) L. Zand et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

C3 glomerulopathy: consensus report

(2013) Matthew C. Pickering et al.   KIDNEY INTERNATIONAL

Toward a working definition of C3 glomerulopathy by immunofluorescence

(2013) Jean Hou et al.   KIDNEY INTERNATIONAL

Dimerization of complement factor H-related proteins modulates complement activation in vivo

(2013) E. Goicoechea de Jorge et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Causes of Alternative Pathway Dysregulation in Dense Deposit Disease

(2012) Y. Zhang et al.   Clinical Journal of the American Society of Nephrology

Eculizumab for Dense Deposit Disease and C3 Glomerulonephritis

(2012) A. S. Bomback et al.   Clinical Journal of the American Society of Nephrology

Combination of Factor H Mutation and Properdin Deficiency Causes Severe C3 Glomerulonephritis

(2012) A. M. Lesher et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

A Hybrid CFHR3-1 Gene Causes Familial C3 Glomerulopathy

(2012) T. H. Malik et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Loss of Properdin Exacerbates C3 Glomerulopathy Resulting from Factor H Deficiency

(2012) M. M. Ruseva et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

C3 glomerulopathy: what's in a name?

(2012) Vivette D. D'Agati et al.   KIDNEY INTERNATIONAL

Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies

(2012) Aude Servais et al.   KIDNEY INTERNATIONAL

Atypical postinfectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement

(2012) Sanjeev Sethi et al.   KIDNEY INTERNATIONAL

Membranoproliferative Glomerulonephritis — A New Look at an Old Entity

(2012) Sanjeev Sethi et al.   NEW ENGLAND JOURNAL OF MEDICINE

Allograft Failure in Kidney Transplant Recipients With Membranoproliferative Glomerulonephritis

(2011) Joseph R. Angelo et al.   AMERICAN JOURNAL OF KIDNEY DISEASES

Familial C3 Glomerulopathy Associated with CFHR5 Mutations: Clinical Characteristics of 91 Patients in 16 Pedigrees

(2011) Y. Athanasiou et al.   Clinical Journal of the American Society of Nephrology

Clinical features and outcomes of 98 children and adults with dense deposit disease

(2011) Der-Fa Lu et al.   PEDIATRIC NEPHROLOGY

Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis

(2010) Daniel P Gale et al.   LANCET

Anti-factor B autoantibody in dense deposit disease

(2010) Stefanie Strobel et al.   MOLECULAR IMMUNOLOGY

Structure of the Extracellular Portion of CD46 Provides Insights into Its Interactions with Complement Proteins and Pathogens

(2010) B. David Persson et al.   PLoS Pathogens

Factor H-related protein 1 (CFHR-1) inhibits complement C5 convertase activity and terminal complex formation

(2009) S. Heinen et al.   BLOOD

Glomeruli of Dense Deposit Disease contain components of the alternative and terminal complement pathway

(2009) Sanjeev Sethi et al.   KIDNEY INTERNATIONAL

Structure of complement fragment C3b–factor H and implications for host protection by complement regulators

(2009) Jin Wu et al.   NATURE IMMUNOLOGY

Dense Deposit Disease: Clinicopathologic Study of 32 Pediatric and Adult Patients

(2008) S. H. Nasr et al.   Clinical Journal of the American Society of Nephrology