Published in Semin Nephrol on November 01, 2013
Complement, a target for therapy in inflammatory and degenerative diseases. Nat Rev Drug Discov (2015) 1.16
Compstatin: a C3-targeted complement inhibitor reaching its prime for bedside intervention. Eur J Clin Invest (2015) 1.08
Update on C3 glomerulopathy. Nephrol Dial Transplant (2014) 0.94
Molecules Great and Small: The Complement System. Clin J Am Soc Nephrol (2015) 0.91
Defining the complement biomarker profile of C3 glomerulopathy. Clin J Am Soc Nephrol (2014) 0.90
Properdin: a tightly regulated critical inflammatory modulator. Immunol Rev (2016) 0.85
Effectiveness of mycophenolate mofetil in C3 glomerulonephritis. Kidney Int (2015) 0.84
Efficacy of Targeted Complement Inhibition in Experimental C3 Glomerulopathy. J Am Soc Nephrol (2015) 0.80
High-Throughput Genetic Testing for Thrombotic Microangiopathies and C3 Glomerulopathies. J Am Soc Nephrol (2015) 0.80
Age-Related Macular Degeneration: A Disease of Systemic or Local Complement Dysregulation? J Clin Med (2014) 0.78
Complement factor H in host defense and immune evasion. Cell Mol Life Sci (2016) 0.78
Reclassification of membranoproliferative glomerulonephritis: Identification of a new GN: C3GN. World J Nephrol (2016) 0.76
Incidence and profile of C3 Glomerulopathy: A single center study. Indian J Nephrol (2015) 0.75
Optimizing the translational value of animal models of glomerulonephritis: insights from recent murine prototypes. Am J Physiol Renal Physiol (2016) 0.75
Protracted Clinical Course of Postinfectious Glomerulonephritis in a Previously Healthy Child. Case Rep Nephrol Dial (2016) 0.75
C3 glomerulopathy and current dilemmas. Clin Exp Nephrol (2016) 0.75
A common haplotype in the complement regulatory gene factor H (HF1/CFH) predisposes individuals to age-related macular degeneration. Proc Natl Acad Sci U S A (2005) 13.44
[Intercapillary deposits of IgA-IgG]. J Urol Nephrol (Paris) (1968) 4.44
Serum C'3 lytic system in patients with glomerulonephritis. Science (1969) 4.44
Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H. Nat Genet (2002) 4.22
Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int (2012) 3.99
A common CFH haplotype, with deletion of CFHR1 and CFHR3, is associated with lower risk of age-related macular degeneration. Nat Genet (2006) 3.96
Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis. Lancet (2010) 3.74
Membranoproliferative glomerulonephritis type II (dense deposit disease): an update. J Am Soc Nephrol (2005) 3.57
Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome. J Med Genet (2006) 3.24
C3 glomerulopathy: a new classification. Nat Rev Nephrol (2010) 3.17
Genome-wide association study identifies susceptibility loci for IgA nephropathy. Nat Genet (2011) 3.15
Glomeruli of Dense Deposit Disease contain components of the alternative and terminal complement pathway. Kidney Int (2009) 3.12
C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int (2012) 3.05
New approaches to the treatment of dense deposit disease. J Am Soc Nephrol (2007) 3.02
Structure and composition of drusen associated with glomerulonephritis: implications for the role of complement activation in drusen biogenesis. Eye (Lond) (2001) 2.85
Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol (2012) 2.83
C3 nephritic factor (C3NeF): stabilization of fluid phase and cell-bound alternative pathway convertase. J Immunol (1976) 2.64
Deletion of Lys224 in regulatory domain 4 of Factor H reveals a novel pathomechanism for dense deposit disease (MPGN II). Kidney Int (2006) 2.64
Spontaneous hemolytic uremic syndrome triggered by complement factor H lacking surface recognition domains. J Exp Med (2007) 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
Proliferative glomerulonephritis with monoclonal IgG deposits. J Am Soc Nephrol (2009) 2.45
The complement abnormalities of lipodystrophy. N Engl J Med (1976) 2.40
Fundus changes in (type II) mesangiocapillary glomerulonephritis simulating drusen: a histopathological report. Br J Ophthalmol (1989) 2.33
C3 glomerulopathy masquerading as acute postinfectious glomerulonephritis. Am J Kidney Dis (2012) 2.28
Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation. J Clin Invest (2010) 2.18
Dense deposit disease: clinicopathologic study of 32 pediatric and adult patients. Clin J Am Soc Nephrol (2008) 2.08
Increased susceptibility to infection associated with abnormalities of complement-mediated functions and of the third component of complement (C3). N Engl J Med (1970) 2.08
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
Severity of primary MPGN, rather than MPGN type, determines renal survival and post-transplantation recurrence risk. Kidney Int (2006) 1.89
The development of atypical hemolytic uremic syndrome depends on complement C5. J Am Soc Nephrol (2010) 1.88
Dense deposit disease is not a membranoproliferative glomerulonephritis. Mod Pathol (2007) 1.88
Prevention of C5 activation ameliorates spontaneous and experimental glomerulonephritis in factor H-deficient mice. Proc Natl Acad Sci U S A (2006) 1.87
Atypical postinfectious glomerulonephritis is associated with abnormalities in the alternative pathway of complement. Kidney Int (2012) 1.81
Eculizumab and refractory membranoproliferative glomerulonephritis. N Engl J Med (2012) 1.80
Complement analysis in children with idiopathic membranoproliferative glomerulonephritis: a long-term follow-up. Pediatr Allergy Immunol (2001) 1.80
Eculizumab for the treatment of dense-deposit disease. N Engl J Med (2012) 1.76
Causes of alternative pathway dysregulation in dense deposit disease. Clin J Am Soc Nephrol (2012) 1.70
Idiopathic mesangiocapillary glomerulonephritis. Comparison of types I and II in children and adults and long-term prognosis. Am J Med (1983) 1.70
Eculizumab in a patient with dense-deposit disease. N Engl J Med (2012) 1.67
Familial C3 glomerulopathy associated with CFHR5 mutations: clinical characteristics of 91 patients in 16 pedigrees. Clin J Am Soc Nephrol (2011) 1.66
Dimerization of complement factor H-related proteins modulates complement activation in vivo. Proc Natl Acad Sci U S A (2013) 1.63
C3 deposition glomerulopathy due to a functional factor H defect. Kidney Int (2008) 1.61
Common polymorphisms in C3, factor B, and factor H collaborate to determine systemic complement activity and disease risk. Proc Natl Acad Sci U S A (2011) 1.60
Dense deposit disease: a variant of membranoproliferative glomerulonephritis. Kidney Int (1975) 1.59
Dense deposit disease associated with monoclonal gammopathy of undetermined significance. Am J Kidney Dis (2010) 1.57
Activation of the alternative pathway of complement by monoclonal lambda light chains in membranoproliferative glomerulonephritis. J Exp Med (1992) 1.56
H deficiency in two brothers with atypical dense intramembranous deposit disease. Kidney Int (1986) 1.55
Clinical features and metabolic and autoimmune derangements in acquired partial lipodystrophy: report of 35 cases and review of the literature. Medicine (Baltimore) (2004) 1.55
A hybrid CFHR3-1 gene causes familial C3 glomerulopathy. J Am Soc Nephrol (2012) 1.52
Nephritogenic lambda light chain dimer: a unique human miniautoantibody against complement factor H. J Immunol (1999) 1.51
Human factor H deficiency. Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism. J Biol Chem (1997) 1.48
Spontaneous clinical improvement in dense deposit disease. Pediatr Nephrol (2000) 1.45
Thrombotic microangiopathy and associated renal disorders. Nephrol Dial Transplant (2012) 1.44
Pathology after eculizumab in dense deposit disease and C3 GN. J Am Soc Nephrol (2012) 1.43
Recurrent dense deposit disease after renal transplantation: an emerging role for complementary therapies. Am J Transplant (2012) 1.40
Combination of factor H mutation and properdin deficiency causes severe C3 glomerulonephritis. J Am Soc Nephrol (2012) 1.40
Allelic variants of complement genes associated with dense deposit disease. J Am Soc Nephrol (2011) 1.38
[DENSE DEPOSITS WITHIN THE BASAL MEMBRANES OF THE KIDNEY. OPTICAL AND ELECTRON MICROSCOPIC STUDY]. Presse Med (1963) 1.34
Loss of properdin exacerbates C3 glomerulopathy resulting from factor H deficiency. J Am Soc Nephrol (2012) 1.34
Linkage of a gene causing familial membranoproliferative glomerulonephritis type III to chromosome 1. J Am Soc Nephrol (2002) 1.31
Glomerulonephritis with isolated C3 deposits and monoclonal gammopathy: a fortuitous association? Clin J Am Soc Nephrol (2011) 1.31
Hypocomplementaemic membranoproliferative glomerulonephritis and nephrotic syndrome associated with partial lipodystrophy of the face and trunk. Proc R Soc Med (1972) 1.31
Dense deposit disease. Mol Immunol (2011) 1.31
Recurrence of membranoproliferative glomerulonephritis type II in renal allografts: The North American Pediatric Renal Transplant Cooperative Study experience. J Am Soc Nephrol (2005) 1.29
Treatment with human complement factor H rapidly reverses renal complement deposition in factor H-deficient mice. Kidney Int (2010) 1.25
C3 glomerulonephritis associated with monoclonal gammopathy: a case series. Am J Kidney Dis (2013) 1.24
Recurrence of complement factor H-related protein 5 nephropathy in a renal transplant. Am J Transplant (2010) 1.16
Renal disease in partial lipodystrophy. Q J Med (1972) 1.16
Composition of nephritic factor-generated glomerular deposits in membranoproliferative glomerulonephritis type 2. Am J Kidney Dis (2001) 1.15
Clinical features and outcomes of 98 children and adults with dense deposit disease. Pediatr Nephrol (2011) 1.13
Genetic deficiency of complement factor H in a patient with age-related macular degeneration and membranoproliferative glomerulonephritis. Mol Immunol (2008) 1.12
Anti-factor B autoantibody in dense deposit disease. Mol Immunol (2010) 1.11
Treatment of mesangiocapillary glomerulonephritis with alternate-day prednisone--a report of the International Study of Kidney Disease in Children. Pediatr Nephrol (1992) 1.10
Factor H facilitates the clearance of GBM bound iC3b by controlling C3 activation in fluid phase. Mol Immunol (2009) 1.09
Inherited factor H deficiency and collagen type III glomerulopathy. Pediatr Nephrol (1995) 1.09
Mesangiocapillary glomerulonephritis type II (dense-deposit disease): clinical features of progressive disease. Am J Kidney Dis (1989) 1.08
In situ complement activation in porcine membranoproliferative glomerulonephritis type II. Kidney Int (1998) 1.07
Tissue-specific host recognition by complement factor H is mediated by differential activities of its glycosaminoglycan-binding regions. J Immunol (2013) 1.06
Phenotypic expression of factor H mutations in patients with atypical hemolytic uremic syndrome. Kidney Int (2006) 1.06
Membranoproliferative glomerulonephritis with disruption of the glomerular basement membrane. Clin Nephrol (1977) 1.04
Acute presentation and persistent glomerulonephritis following streptococcal infection in a patient with heterozygous complement factor H-related protein 5 deficiency. Am J Kidney Dis (2012) 1.03
Disruption of disulfide bonds is responsible for impaired secretion in human complement factor H deficiency. J Biol Chem (1999) 1.02
Hereditary complement factor I deficiency. QJM (1994) 1.02
Effect of nephritic factor on C3 and on the terminal pathway of complement in vivo and in vitro. Clin Exp Immunol (1986) 1.01
Heterogeneous pattern of renal disease associated with homozygous factor H deficiency. Hum Pathol (2011) 1.00
[2 cases of membrano-proliferative glomerulonephritis associated with partial lipodistrophy of facio-truncal type]. J Urol Nephrol (Paris) (1970) 1.00
Dense intramembranous deposit disease: a clinical comparison of histological subtypes. Clin Nephrol (1990) 0.99
Familial membranoproliferative glomerulonephritis type III. Am J Kidney Dis (2002) 0.97
Dense deposit disease in association with monoclonal gammopathy of unknown significance. Nephrol Dial Transplant (1996) 0.97
Prognosis, treatment and outcome of childhood mesangiocapillary (membranoproliferative) glomerulonephritis. Nephrol Dial Transplant (2004) 0.97
An inherited defect in the C3 convertase, C3b,Bb, associated with glomerulonephritis. Kidney Int (1983) 0.96
Renal transplantation in patients with dense deposit disease: morphological characteristics of recurrent disease and clinical outcome. Nephrol Dial Transplant (1999) 0.95
The molecular basis for hereditary porcine membranoproliferative glomerulonephritis type II: point mutations in the factor H coding sequence block protein secretion. Am J Pathol (2002) 0.95
Hypocomplementemic glomerulonephritis in an infant and mother. Evidence for an abnormal form of C3. Am J Nephrol (1987) 0.94
Regression of membranoproliferative glomerulonephritis type II (dense deposit disease): observations in six children. Am J Kidney Dis (1988) 0.94
Mesangiocapillary glomerulonephritis: a long-term study of 40 cases. J Pathol (1983) 0.93
Electron dense alteration of kidney basement membranes. A renal lesion specific of a systemic disease. Am J Med (1975) 0.93
Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis. Lancet (2010) 3.74
C3 glomerulopathy: a new classification. Nat Rev Nephrol (2010) 3.17
New approaches to the treatment of dense deposit disease. J Am Soc Nephrol (2007) 3.02
Detection of glomerular complement C3 fragments by magnetic resonance imaging in murine lupus nephritis. Kidney Int (2011) 2.20
Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient mice. J Clin Invest (2008) 1.94
The development of atypical hemolytic uremic syndrome depends on complement C5. J Am Soc Nephrol (2010) 1.88
C1q deficiency and autoimmunity: the effects of genetic background on disease expression. J Immunol (2002) 1.88
Complement in human diseases: Lessons from complement deficiencies. Mol Immunol (2009) 1.73
Dimerization of complement factor H-related proteins modulates complement activation in vivo. Proc Natl Acad Sci U S A (2013) 1.63
Complement factor H deficiency in aged mice causes retinal abnormalities and visual dysfunction. Proc Natl Acad Sci U S A (2007) 1.62
A hybrid CFHR3-1 gene causes familial C3 glomerulopathy. J Am Soc Nephrol (2012) 1.52
C3 glomerulopathy-associated CFHR1 mutation alters FHR oligomerization and complement regulation. J Clin Invest (2013) 1.52
Atypical hemolytic uremic syndrome and genetic aberrations in the complement factor H-related 5 gene. J Hum Genet (2012) 1.46
Dense deposit disease. Mol Immunol (2011) 1.31
Treatment with human complement factor H rapidly reverses renal complement deposition in factor H-deficient mice. Kidney Int (2010) 1.25
Recent insights into C3 glomerulopathy. Nephrol Dial Transplant (2013) 1.21
C3 glomerulopathy: clinicopathologic features and predictors of outcome. Clin J Am Soc Nephrol (2013) 1.19
The follicular dendritic cell restricted epitope, FDC-M2, is complement C4; localization of immune complexes in mouse tissues. Eur J Immunol (2002) 1.19
Design and evaluation of meningococcal vaccines through structure-based modification of host and pathogen molecules. PLoS Pathog (2012) 1.10
Factor H facilitates the clearance of GBM bound iC3b by controlling C3 activation in fluid phase. Mol Immunol (2009) 1.09
P2X7 deficiency attenuates renal injury in experimental glomerulonephritis. J Am Soc Nephrol (2009) 1.08
Acute presentation and persistent glomerulonephritis following streptococcal infection in a patient with heterozygous complement factor H-related protein 5 deficiency. Am J Kidney Dis (2012) 1.03
Phagocytosis is the main CR3-mediated function affected by the lupus-associated variant of CD11b in human myeloid cells. PLoS One (2013) 1.01
Binding of factor H to tubular epithelial cells limits interstitial complement activation in ischemic injury. Kidney Int (2011) 1.00
Decay-accelerating factor suppresses complement C3 activation and retards atherosclerosis in low-density lipoprotein receptor-deficient mice. Am J Pathol (2009) 0.96
Crry deficiency in complement sufficient mice: C3 consumption occurs without associated renal injury. Mol Immunol (2008) 0.96
Complement factor h limits immune complex deposition and prevents inflammation and scarring in glomeruli of mice with chronic serum sickness. J Am Soc Nephrol (2004) 0.95
Nonfunctional variant 3 factor H binding proteins as meningococcal vaccine candidates. Infect Immun (2013) 0.95
Detection of complement activation using monoclonal antibodies against C3d. J Clin Invest (2013) 0.93
Complement component C3 plays a critical role in protecting the aging retina in a murine model of age-related macular degeneration. Am J Pathol (2013) 0.91
Successful treatment of acute haemorrhagic cytomegalovirus colitis with ganciclovir in an individual without overt immunocompromise. Eur J Gastroenterol Hepatol (2003) 0.87
Regulating complement in the kidney: insights from CFHR5 nephropathy. Dis Model Mech (2011) 0.87
Essential role of surface-bound complement factor H in controlling immune complex-induced arthritis. J Immunol (2013) 0.87
SLE with C1q deficiency treated with fresh frozen plasma: a 10-year experience. Rheumatology (Oxford) (2009) 0.83
Experimental models of membranoproliferative glomerulonephritis, including dense deposit disease. Contrib Nephrol (2011) 0.81
Relationship between complotype and reported severity of systemic allergic reactions to peanut. J Allergy Clin Immunol (2012) 0.78
Atypical hemolytic uremic syndrome: telling the difference between H and Y. Kidney Int (2010) 0.76
Abnormally High Content of Free Glucosamine Residues Identified in a Preparation of Commercially Available Porcine Intestinal Heparan Sulfate. Anal Chem (2016) 0.75
Lateral medullary syndrome with anti-neuronal antibodies (anti-Ta/Ma2) in primary Sjogren's syndrome. Rheumatology (Oxford) (2009) 0.75
Painful myositis in the anti-synthetase syndrome with anti-PL12 antibodies. Rheumatol Int (2011) 0.75