Published in J Med Genet on October 03, 2006
Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis. Lancet (2010) 3.74
A rare penetrant mutation in CFH confers high risk of age-related macular degeneration. Nat Genet (2011) 3.55
C3 glomerulopathy: a new classification. Nat Rev Nephrol (2010) 3.17
C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int (2012) 3.05
Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol (2012) 2.83
Membranoproliferative glomerulonephritis and C3 glomerulopathy: resolving the confusion. Kidney Int (2011) 2.61
Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient mice. J Clin Invest (2008) 1.94
Proliferative glomerulonephritis secondary to dysfunction of the alternative pathway of complement. Clin J Am Soc Nephrol (2011) 1.92
Translational mini-review series on complement factor H: renal diseases associated with complement factor H: novel insights from humans and animals. Clin Exp Immunol (2008) 1.89
Atypical postinfectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement. Kidney Int (2012) 1.81
Familial C3 glomerulopathy associated with CFHR5 mutations: clinical characteristics of 91 patients in 16 pedigrees. Clin J Am Soc Nephrol (2011) 1.66
Pathogenesis of the C3 glomerulopathies and reclassification of MPGN. Nat Rev Nephrol (2012) 1.58
Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome. Blood (2007) 1.53
Pathology after eculizumab in dense deposit disease and C3 GN. J Am Soc Nephrol (2012) 1.43
Combination of factor H mutation and properdin deficiency causes severe C3 glomerulonephritis. J Am Soc Nephrol (2012) 1.40
Glomerulonephritis with isolated C3 deposits and monoclonal gammopathy: a fortuitous association? Clin J Am Soc Nephrol (2011) 1.31
C3 glomerulonephritis associated with monoclonal gammopathy: a case series. Am J Kidney Dis (2013) 1.24
Recent insights into C3 glomerulopathy. Nephrol Dial Transplant (2013) 1.21
Recurrence of complement factor H-related protein 5 nephropathy in a renal transplant. Am J Transplant (2010) 1.16
Structural basis for complement factor I control and its disease-associated sequence polymorphisms. Proc Natl Acad Sci U S A (2011) 1.12
Mayo Clinic/Renal Pathology Society Consensus Report on Pathologic Classification, Diagnosis, and Reporting of GN. J Am Soc Nephrol (2015) 1.08
Treatment options for C3 glomerulopathy. Curr Opin Nephrol Hypertens (2013) 1.07
The role of molecular genetics in diagnosing familial hematuria(s). Pediatr Nephrol (2011) 1.07
Eculizumab and recurrent C3 glomerulonephritis. Pediatr Nephrol (2013) 1.07
Membranoproliferative glomerulonephritis with C3NeF and genetic complement dysregulation. Pediatr Nephrol (2010) 1.05
Association of a novel complement factor H mutation with severe crescentic and necrotizing glomerulonephritis. Am J Kidney Dis (2012) 1.03
C4d as a Diagnostic Tool in Proliferative GN. J Am Soc Nephrol (2015) 1.03
Current Understanding of the Role of Complement in IgA Nephropathy. J Am Soc Nephrol (2015) 0.99
Dense deposit disease and C3 glomerulopathy. Semin Nephrol (2013) 0.98
New functional and structural insights from updated mutational databases for complement factor H, Factor I, membrane cofactor protein and C3. Biosci Rep (2014) 0.96
Clinical findings, pathology, and outcomes of C3GN after kidney transplantation. J Am Soc Nephrol (2013) 0.95
Properdin in complement activation and tissue injury. Mol Immunol (2013) 0.95
Inhibiting complement activation on cells at the step of C3 cleavage. Vaccine (2008) 0.94
C3 nephritic factor associated with C3 glomerulopathy in children. Pediatr Nephrol (2013) 0.91
Secondary focal and segmental glomerulosclerosis associated with single-nucleotide polymorphisms in the genes encoding complement factor H and C3. Am J Kidney Dis (2012) 0.91
Defective complement inhibitory function predisposes to renal disease. Annu Rev Med (2012) 0.89
Mutations in genes encoding complement inhibitors CD46 and CFH affect the age at nephritis onset in patients with systemic lupus erythematosus. Arthritis Res Ther (2011) 0.87
Regulating complement in the kidney: insights from CFHR5 nephropathy. Dis Model Mech (2011) 0.87
Neutrophils: game changers in glomerulonephritis? Trends Mol Med (2010) 0.86
Effectiveness of mycophenolate mofetil in C3 glomerulonephritis. Kidney Int (2015) 0.84
Complement regulator CD46: genetic variants and disease associations. Hum Genomics (2015) 0.82
Molecular Basis of Factor H R1210C Association with Ocular and Renal Diseases. J Am Soc Nephrol (2015) 0.82
Paraprotein-Related Kidney Disease: Glomerular Diseases Associated with Paraproteinemias. Clin J Am Soc Nephrol (2016) 0.81
An emerging molecular understanding and novel targeted treatment approaches in pediatric kidney diseases. Front Pediatr (2014) 0.81
Efficacy of Targeted Complement Inhibition in Experimental C3 Glomerulopathy. J Am Soc Nephrol (2015) 0.80
American Society of Nephrology Quiz and Questionnaire 2012: glomerulonephritis. Clin J Am Soc Nephrol (2013) 0.80
Complement involvement in kidney diseases: From physiopathology to therapeutical targeting. World J Nephrol (2015) 0.80
C1q nephropathy in the pediatric population: pathology and pathogenesis. Pediatr Nephrol (2010) 0.79
C3 mesangial proliferative glomerulonephritis initially presenting with atypical hemolytic uremic syndrome: a case report. J Med Case Rep (2016) 0.79
Nanosized contrast agents to noninvasively detect kidney inflammation by magnetic resonance imaging. Adv Chronic Kidney Dis (2013) 0.78
Temporal and Demographic Trends in Glomerular Disease Epidemiology in the Southeastern United States, 1986-2015. Clin J Am Soc Nephrol (2017) 0.78
Partial Complement Factor H Deficiency Associates with C3 Glomerulopathy and Thrombotic Microangiopathy. J Am Soc Nephrol (2015) 0.78
Complement factor H mutation associated with membranoproliferative glomerulonephritis with transformation to atypical haemolytic uraemic syndrome. Clin Kidney J (2013) 0.78
Update on membranoproliferative GN. Clin J Am Soc Nephrol (2014) 0.78
Anti-glomerular basement membrane glomerulonephritis and thrombotic microangiopathy in first degree relatives: a case report. BMC Nephrol (2012) 0.77
Complement factor H R1210C among Japanese patients with age-related macular degeneration. Jpn J Ophthalmol (2015) 0.77
Reclassification of membranoproliferative glomerulonephritis: Identification of a new GN: C3GN. World J Nephrol (2016) 0.76
The identification of CFHR5 nephropathy. J R Soc Med (2011) 0.76
Distinct roles for the complement regulators factor H and Crry in protection of the kidney from injury. Kidney Int (2016) 0.76
Making sense of the spectrum of glomerular disease associated with complement dysregulation. Pediatr Nephrol (2013) 0.76
Overview of C3 Glomerulopathy. Front Pediatr (2016) 0.75
Incidence and profile of C3 Glomerulopathy: A single center study. Indian J Nephrol (2015) 0.75
A rare case: childhood-onset C3 glomerulonephritis due to homozygous factor H deficiency. CEN Case Rep (2013) 0.75
Different types of glomerulonephritis associated with the dysregulation of the complement alternative pathway in 2 brothers: A case report. Medicine (Baltimore) (2017) 0.75
C3 glomerulopathy in adults: a distinct patient subset showing frequent association with monoclonal gammopathy and poor renal outcome. Clin Kidney J (2016) 0.75
Clinico-pathologic spectrum of C3 glomerulopathy-an Indian experience. Diagn Pathol (2015) 0.75
New classification of membranoproliferative glomerulonephritis: a good start but a long way to go. Kidney Res Clin Pract (2014) 0.75
Reanalysis of membranoproliferative glomerulonephritis patients according to the new classification: a multicenter study. Kidney Res Clin Pract (2014) 0.75
Current concepts in C3 glomerulopathy. Indian J Nephrol (2014) 0.75
Decade in review-glomerular disease: The glomerulus reveals some secrets. Nat Rev Nephrol (2015) 0.75
C3 glomerulopathy and current dilemmas. Clin Exp Nephrol (2016) 0.75
Novel factor H mutation associated with familial membranoproliferative glomerulonephritis type I. Pediatr Nephrol (2015) 0.75
K/DOQI clinical practice guidelines for chronic kidney disease: evaluation, classification, and stratification. Am J Kidney Dis (2002) 81.62
Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood (2006) 4.51
Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H. Nat Genet (2002) 4.22
Genetic studies into inherited and sporadic hemolytic uremic syndrome. Kidney Int (1998) 4.03
Membranoproliferative glomerulonephritis type II (dense deposit disease): an update. J Am Soc Nephrol (2005) 3.57
Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol (2004) 2.97
Mutations in factor H reduce binding affinity to C3b and heparin and surface attachment to endothelial cells in hemolytic uremic syndrome. J Clin Invest (2003) 2.96
Familial haemolytic uraemic syndrome and an MCP mutation. Lancet (2003) 2.79
Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome. Proc Natl Acad Sci U S A (2003) 2.79
Clustering of missense mutations in the C-terminal region of factor H in atypical hemolytic uremic syndrome. Am J Hum Genet (2001) 2.75
The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20. J Am Soc Nephrol (2001) 2.56
Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome. J Med Genet (2004) 2.55
Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases. J Am Soc Nephrol (2004) 2.46
Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome. J Am Soc Nephrol (2005) 2.23
Haemolytic uraemic syndrome and mutations of the factor H gene: a registry-based study of German speaking countries. J Med Genet (2003) 1.92
Prevention of C5 activation ameliorates spontaneous and experimental glomerulonephritis in factor H-deficient mice. Proc Natl Acad Sci U S A (2006) 1.87
Implications of the initial mutations in membrane cofactor protein (MCP; CD46) leading to atypical hemolytic uremic syndrome. Mol Immunol (2006) 1.82
Hypocomplementaemia of poststreptococcal acute glomerulonephritis is associated with C3 nephritic factor (C3NeF) IgG autoantibody activity. Nephrol Dial Transplant (1994) 1.80
Complement analysis in children with idiopathic membranoproliferative glomerulonephritis: a long-term follow-up. Pediatr Allergy Immunol (2001) 1.80
Nephritis-associated plasmin receptor and acute poststreptococcal glomerulonephritis: characterization of the antigen and associated immune response. J Am Soc Nephrol (2004) 1.67
Is the nephritogenic antigen in post-streptococcal glomerulonephritis pyrogenic exotoxin B (SPE B) or GAPDH? Kidney Int (2005) 1.67
Factor H and atypical hemolytic uremic syndrome: mutations in the C-terminus cause structural changes and defective recognition functions. J Am Soc Nephrol (2005) 1.62
H deficiency in two brothers with atypical dense intramembranous deposit disease. Kidney Int (1986) 1.55
Membrane cofactor protein: importance of N- and O-glycosylation for complement regulatory function. J Immunol (1998) 1.30
Immunohistochemical and serological evidence for the role of streptococcal proteinase in acute post-streptococcal glomerulonephritis. Kidney Int (1998) 1.10
Inherited complement component deficiencies in membranoproliferative glomerulonephritis. Kidney Int (1983) 1.04
Complement factor I deficiency associated with recurrent infections, vasculitis and immune complex glomerulonephritis. Scand J Infect Dis (2005) 0.97
Secondary membranoproliferative glomerulonephritis due to hemolytic uremic syndrome: an unusual presentation. Ren Fail (1998) 0.91
Recurrent glomerulonephritis after renal transplantation. Annu Rev Med (1978) 0.90
The classification of glomerulonephritis in systemic lupus erythematosus revisited. J Am Soc Nephrol (2004) 7.38
Complement-binding anti-HLA antibodies and kidney-allograft survival. N Engl J Med (2013) 6.31
Inhibition of the mTORC pathway in the antiphospholipid syndrome. N Engl J Med (2014) 5.33
The classification of glomerulonephritis in systemic lupus erythematosus revisited. Kidney Int (2004) 4.77
The ciliary gene RPGRIP1L is mutated in cerebello-oculo-renal syndrome (Joubert syndrome type B) and Meckel syndrome. Nat Genet (2007) 4.63
Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int (2012) 3.99
Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis. Lancet (2010) 3.74
Histopathologic classification of ANCA-associated glomerulonephritis. J Am Soc Nephrol (2010) 3.48
Pleiotropic effects of CEP290 (NPHP6) mutations extend to Meckel syndrome. Am J Hum Genet (2007) 3.42
Atypical hemolytic uremic syndrome. Orphanet J Rare Dis (2011) 3.35
Clinical spectrum associated with hepatocyte nuclear factor-1beta mutations. Ann Intern Med (2004) 3.32
Octogenarians reaching end-stage renal disease: cohort study of decision-making and clinical outcomes. J Am Soc Nephrol (2003) 3.25
C3 glomerulopathy: a new classification. Nat Rev Nephrol (2010) 3.17
Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome. Blood (2008) 3.10
Segregation at three loci explains familial and population risk in Hirschsprung disease. Nat Genet (2002) 3.07
Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol (2004) 2.97
Recessive mutations in DGKE cause atypical hemolytic-uremic syndrome. Nat Genet (2013) 2.91
Genetic and functional analyses of membrane cofactor protein (CD46) mutations in atypical hemolytic uremic syndrome. J Am Soc Nephrol (2006) 2.88
C3 glomerulopathy: consensus report. Kidney Int (2013) 2.85
Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies. Nat Rev Nephrol (2012) 2.62
Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults. Clin J Am Soc Nephrol (2013) 2.47
Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases. J Am Soc Nephrol (2004) 2.46
FGF9 and FGF20 maintain the stemness of nephron progenitors in mice and man. Dev Cell (2012) 2.43
Cyst infections in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol (2009) 2.42
The gene mutated in juvenile nephronophthisis type 4 encodes a novel protein that interacts with nephrocystin. Nat Genet (2002) 2.41
Clinical and epidemiological assessment of steroid-resistant nephrotic syndrome associated with the NPHS2 R229Q variant. Kidney Int (2009) 2.40
Early angiotensin-converting enzyme inhibition in Alport syndrome delays renal failure and improves life expectancy. Kidney Int (2011) 2.34
Hyperfunctional C3 convertase leads to complement deposition on endothelial cells and contributes to atypical hemolytic uremic syndrome. Blood (2009) 2.33
Lipocalin 2 is essential for chronic kidney disease progression in mice and humans. J Clin Invest (2010) 2.23
Post-allogeneic haematopoietic stem cell transplantation membranous nephropathy: clinical presentation, outcome and pathogenic aspects. Nephrol Dial Transplant (2007) 2.17
X-linked Alport syndrome: natural history and genotype-phenotype correlations in girls and women belonging to 195 families: a "European Community Alport Syndrome Concerted Action" study. J Am Soc Nephrol (2003) 2.12
High NPHP1 and NPHP6 mutation rate in patients with Joubert syndrome and nephronophthisis: potential epistatic effect of NPHP6 and AHI1 mutations in patients with NPHP1 mutations. J Am Soc Nephrol (2007) 2.06
The clinical spectrum and therapeutic management of hypocomplementemic urticarial vasculitis: data from a French nationwide study of fifty-seven patients. Arthritis Rheumatol (2015) 1.98
Rab35 GTPase and OCRL phosphatase remodel lipids and F-actin for successful cytokinesis. Nat Cell Biol (2011) 1.96
High sirolimus levels may induce focal segmental glomerulosclerosis de novo. Clin J Am Soc Nephrol (2007) 1.90
ANCA-negative pauci-immune renal vasculitis: histology and outcome. Nephrol Dial Transplant (2005) 1.84
Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: A prospective analysis of 100 patients with severe renal involvement. J Am Soc Nephrol (2006) 1.82
Expression of the nonmuscle myosin heavy chain IIA in the human kidney and screening for MYH9 mutations in Epstein and Fechtner syndromes. J Am Soc Nephrol (2002) 1.79
Renal histology in ANCA-associated vasculitis: differences between diagnostic and serologic subgroups. Kidney Int (2002) 1.78
Characterization of the nephrocystin/nephrocystin-4 complex and subcellular localization of nephrocystin-4 to primary cilia and centrosomes. Hum Mol Genet (2005) 1.77
Thrombotic microangiopathy secondary to VEGF pathway inhibition by sunitinib. Nephrol Dial Transplant (2008) 1.76
Fanconi syndrome due to deferasirox. Am J Kidney Dis (2009) 1.61
Enzyme replacement therapy in Anderson-Fabry's disease: beneficial clinical effect on vital organ function. Nephrol Dial Transplant (2003) 1.60
The interactive Factor H-atypical hemolytic uremic syndrome mutation database and website: update and integration of membrane cofactor protein and Factor I mutations with structural models. Hum Mutat (2007) 1.59
Mutations in components of complement influence the outcome of Factor I-associated atypical hemolytic uremic syndrome. Kidney Int (2009) 1.55
Clinical features of anti-factor H autoantibody-associated hemolytic uremic syndrome. J Am Soc Nephrol (2010) 1.53
Clinical features and management of arterial hypertension in children with Williams-Beuren syndrome. Nephrol Dial Transplant (2009) 1.51
A clinicopathologic study of thrombotic microangiopathy in IgA nephropathy. J Am Soc Nephrol (2011) 1.51
Defects in the IFT-B component IFT172 cause Jeune and Mainzer-Saldino syndromes in humans. Am J Hum Genet (2013) 1.51
Elevated soluble Flt1 inhibits endothelial repair in PR3-ANCA-associated vasculitis. J Am Soc Nephrol (2011) 1.50
Lithium-induced nephropathy: Rate of progression and prognostic factors. Kidney Int (2003) 1.48
Familial ATTR amyloidosis: microalbuminuria as a predictor of symptomatic disease and clinical nephropathy. Nephrol Dial Transplant (2003) 1.48
CC2D2A mutations in Meckel and Joubert syndromes indicate a genotype-phenotype correlation. Hum Mutat (2009) 1.48
Determinants of outcome in ANCA-associated glomerulonephritis: a prospective clinico-histopathological analysis of 96 patients. Kidney Int (2002) 1.44
Renal involvement in monoclonal (type I) cryoglobulinemia: two cases associated with IgG3 kappa cryoglobulin. Am J Kidney Dis (2002) 1.44
Renal lesions associated with IgM-secreting monoclonal proliferations: revisiting the disease spectrum. Clin J Am Soc Nephrol (2008) 1.43
Prevalence of monoclonal gammopathy in patients presenting with acquired angioedema type 2. Am J Med (2002) 1.42
Cysteamine therapy delays the progression of nephropathic cystinosis in late adolescents and adults. Kidney Int (2011) 1.39
Nephrocystin-1 and nephrocystin-4 are required for epithelial morphogenesis and associate with PALS1/PATJ and Par6. Hum Mol Genet (2009) 1.37
New insights into postrenal transplant hemolytic uremic syndrome. Nat Rev Nephrol (2010) 1.36
Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases. Blood (2005) 1.32
Spectrum of HNF1B mutations in a large cohort of patients who harbor renal diseases. Clin J Am Soc Nephrol (2010) 1.32
Glomerulonephritis with isolated C3 deposits and monoclonal gammopathy: a fortuitous association? Clin J Am Soc Nephrol (2011) 1.31
Complement alternative pathway acts as a positive feedback amplification of neutrophil activation. Blood (2010) 1.31
Anti-CTLA4 antibody-induced lupus nephritis. N Engl J Med (2009) 1.30
Phenotype and genotype characterization of adenine phosphoribosyltransferase deficiency. J Am Soc Nephrol (2010) 1.30
SLE and idiopathic nephrotic syndrome: coincidence or not? Am J Kidney Dis (2002) 1.29
Genetic investigation of autosomal recessive distal renal tubular acidosis: evidence for early sensorineural hearing loss associated with mutations in the ATP6V0A4 gene. J Am Soc Nephrol (2006) 1.29
Steroid-sensitive nephrotic syndrome: from childhood to adulthood. Am J Kidney Dis (2003) 1.28