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1. Full Text Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies
by Servais, Aude and Noël, Laure-Hélène and Roumenina, Lubka T and Le Quintrec, Moglie and Ngo, Stephanie and Dragon-Durey, Marie-Agnès and Macher, Marie-Alice and Zuber, Julien and Karras, Alexandre and Provot, François and Moulin, Bruno and Grünfeld, Jean-Pierre and Niaudet, Patrick and Lesavre, Philippe and Frémeaux-Bacchi, Véronique
Kidney International, ISSN 0085-2538, 08/2012, Volume 82, Issue 4, pp. 454 - 464
Dense deposit disease and glomerulonephritis with isolated C3 deposits are glomerulopathies characterized by deposits of C3 within or along the glomerular... 
complement | glomerulonephritis | clinical immunology | membranoproliferative glomerulonephritis (MPGN) | LONG-TERM | HEMOLYTIC-UREMIC SYNDROME | MESANGIOCAPILLARY GLOMERULONEPHRITIS | GLOMERULONEPHRITIS TYPE-II | MACULAR DEGENERATION | FACTOR-H DEFICIENCY | GLOMERULAR-FILTRATION-RATE | UROLOGY & NEPHROLOGY | MUTATIONS | MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS | MEMBRANE COFACTOR PROTEIN | Humans | Child, Preschool | Male | Complement System Proteins - metabolism | Young Adult | Time Factors | DNA Mutational Analysis | Renal Insufficiency - immunology | Child | Glomerulonephritis, Membranoproliferative - pathology | Genetic Predisposition to Disease | Risk Assessment | Complement C3 - metabolism | Gene Frequency | Risk Factors | Glomerulonephritis - genetics | Glomerulonephritis - immunology | Glomerulonephritis - mortality | Biomarkers - blood | Kidney Glomerulus - pathology | Disease Progression | Glomerulonephritis, Membranoproliferative - genetics | Glomerulonephritis, Membranoproliferative - immunology | Phenotype | Complement Factor H - metabolism | Glomerulonephritis, Membranoproliferative - mortality | Glomerulonephritis - pathology | Adolescent | Age of Onset | Membrane Cofactor Protein - genetics | Kidney Glomerulus - immunology | Mutation | Complement Factor I - metabolism | Haplotypes | Complement C3 Nephritic Factor - genetics | Glomerulonephritis - therapy | Infant | Case-Control Studies | Adult | Complement System Proteins - genetics | Female | Membrane Cofactor Protein - metabolism | Complement Pathway, Alternative - genetics | France | Renal Insufficiency - genetics | Kaplan-Meier Estimate | Complement Factor I - genetics | Treatment Outcome | Chi-Square Distribution | Glomerulonephritis, Membranoproliferative - therapy | Biopsy | Complement C3 Nephritic Factor - metabolism | Complement Factor H - genetics
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2. Full Text C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up
by Sethi, Sanjeev and Fervenza, Fernando C and Zhang, Yuzhou and Zand, Ladan and Vrana, Julie A and Nasr, Samih H and Theis, Jason D and Dogan, Ahmet and Smith, Richard J.H
Kidney International, ISSN 0085-2538, 08/2012, Volume 82, Issue 4, pp. 465 - 473
C3 glomerulonephritis (C3GN) is a recently described disorder that typically results from abnormalities in the alternative pathway (AP) of complement. Here, we... 
C3GN | proteomics | C3 glomerulopathy | MPGN | alternative pathway of complement | C3 glomerulonephritis | MACULAR DEGENERATION | CLASSIFICATION | RISK | PROTEIN 5 | DENSE DEPOSIT DISEASE | ALTERNATIVE PATHWAY | UROLOGY & NEPHROLOGY | MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS | Complement C3 - analysis | Predictive Value of Tests | Recurrence | Follow-Up Studies | Humans | Middle Aged | Complement System Proteins - immunology | Laser Capture Microdissection | Molecular Sequence Data | Male | Glomerulonephritis, Membranoproliferative - complications | Complement System Proteins - analysis | Young Adult | Time Factors | DNA Mutational Analysis | Mass Spectrometry | Adult | Autoantibodies - analysis | Complement System Proteins - genetics | Female | Complement Pathway, Alternative - genetics | Proteomics - methods | Child | Amino Acid Sequence | Glomerulonephritis, Membranoproliferative - pathology | Genetic Predisposition to Disease | Proteinuria - immunology | Treatment Outcome | Complement C3 Nephritic Factor - analysis | Hematuria - immunology | Kidney Transplantation | Minnesota | Kidney Glomerulus - pathology | Glomerulonephritis, Membranoproliferative - therapy | Glomerulonephritis, Membranoproliferative - genetics | Glomerulonephritis, Membranoproliferative - immunology | Biopsy | Adolescent | Aged | Kidney Glomerulus - immunology | Mutation | Complement Factor H - genetics
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3. Full Text Pathogenesis of the C3 glomerulopathies and reclassification of MPGN
by Bomback, Andrew S and Appel, Gerald B
Nature Reviews Nephrology, ISSN 1759-5061, 11/2012, Volume 8, Issue 11, pp. 634 - 642
Until recently, membranoproliferative glomerulonephritis (MPGN) was clinically classified as either primary, idiopathic MPGN or as secondary MPGN when an... 
COMPLEMENT FACTOR-H | DENSE DEPOSIT DISEASE | NEPHRITIC FACTOR | HYPOCOMPLEMENTEMIA | INHIBITOR ECULIZUMAB | ALTERNATIVE PATHWAY | ABNORMALITIES | DYSREGULATION | UROLOGY & NEPHROLOGY | MONOCLONAL GAMMOPATHY | MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS | Glomerulonephritis, Membranoproliferative - genetics | Glomerulonephritis, Membranoproliferative - pathology | Glomerulonephritis, Membranoproliferative - immunology | Glomerulonephritis, Membranoproliferative - classification | Prognosis | Kidney - pathology | Complement Pathway, Alternative - immunology | Humans | Complement System Proteins - immunology | Glomerulonephritis, Membranoproliferative - diagnosis | Complement Activation - immunology | Complement C3 - immunology | Glomerulonephritis | Care and treatment | Diagnosis | Complement (Immunology) | Health aspects | Identification and classification
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4. Full Text Glomerulonephritis With Isolated C3 Deposits and Monoclonal Gammopathy: A Fortuitous Association?
by Frank Bridoux and Estelle Desport and Véronique Frémeaux-Bacchi and Christine Fen Chong and Jean-Marc Gombert and Corinne Lacombe and Nathalie Quellard and Guy Touchard
Clinical Journal of the American Society of Nephrology, ISSN 1555-9041, 09/2011, Volume 6, Issue 9, pp. 2165 - 2174
Glomerular deposition of monoclonal Ig has been exceptionally described as the cause of membranoproliferative glomerulonephritis, through activation of the... 
Complement C3 - analysis | Glomerulonephritis, Membranoproliferative - pathology | Follow-Up Studies | Humans | Middle Aged | Male | Paraproteinemias - complications | Glomerulonephritis, Membranoproliferative - therapy | Glomerulonephritis, Membranoproliferative - immunology | Adult | Female | Aged | Retrospective Studies | Complement Pathway, Alternative | Glomerulonephritis, Membranoproliferative - etiology | Complement C3 | Life Sciences | Glomerulonephritis, Membranoproliferative | Immunology | Paraproteinemias | Original
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5. Full Text The role of complement in C3 glomerulopathy
by Zipfel, Peter F and Skerka, Christine and Chen, Qian and Wiech, Thorsten and Goodship, Tim and Johnson, Sally and Fremeaux-Bacchi, Veronique and Nester, Clara and Córdoba, Santiago Rodríguez de and Noris, Marina and Pickering, Matthew and Smith, Richard
Molecular Immunology, ISSN 0161-5890, 09/2015, Volume 67, Issue 1, pp. 21 - 30
C3 glomerulopathy describes a spectrum of disorders with glomerular pathology associated with C3 cleavage product deposition and with defective complement... 
MPGN membranoproliferative | C3 nephritic factor | Dense deposit diseases | Complement C3 glomerulopathy | HEMOLYTIC-UREMIC SYNDROME | BIOCHEMISTRY & MOLECULAR BIOLOGY | GLOMERULONEPHRITIS TYPE-II | MACULAR DEGENERATION | IMMUNOLOGY | FACTOR-H-AUTOANTIBODIES | DENSE DEPOSIT DISEASE | NEPHRITIC FACTOR | ALTERNATIVE PATHWAY | BINDING-AFFINITY | NEISSERIA-GONORRHOEAE | MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS | Complement C3 Nephritic Factor - genetics | Complement Activation | Humans | Complement C3b Inactivator Proteins - genetics | Complement C3b Inactivator Proteins - chemistry | Complement C3 - chemistry | Protein Aggregation, Pathological - pathology | Protein Aggregation, Pathological - diagnosis | B-Lymphocytes - pathology | Protein Aggregation, Pathological - immunology | T-Lymphocytes - pathology | Complement C3 - genetics | Protein Aggregation, Pathological - genetics | Adaptive Immunity | Glomerulonephritis, Membranoproliferative - pathology | Gene Expression | Complement C3 Nephritic Factor - chemistry | Biomarkers - blood | Kidney Glomerulus - pathology | Glomerulonephritis, Membranoproliferative - genetics | Glomerulonephritis, Membranoproliferative - immunology | B-Lymphocytes - immunology | T-Lymphocytes - immunology | Glomerulonephritis, Membranoproliferative - diagnosis | Kidney Glomerulus - immunology | Kidney Glomerulus - chemistry | Medical colleges
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6. Full Text Clinical findings, pathology, and outcomes of C3GN after kidney transplantation
by Zand, Ladan and Zand, Ladan and Lorenz, Elizabeth C and Lorenz, Elizabeth C and Cosio, Fernando G and Cosio, Fernando G and Fervenza, Fernando C and Fervenza, Fernando C and Nasr, Samih H and Nasr, Samih H and Gandhi, Manish J and Gandhi, Manish J and Smith, Richard J.H and Smith, Richard J H and Sethi, Sanjeev and Sethi, Sanjeev
Journal of the American Society of Nephrology : JASN, ISSN 1046-6673, 2014, Volume 25, Issue 5, pp. 1110 - 1117
C3 glomerulonephritis (C3GN) results from abnormalities in the alternative pathway of complement, and it is characterized by deposition of C3 with absent or... 
DENSE DEPOSIT DISEASE | COMPLEMENT | GLOMERULOPATHY | ENTITY | ALTERNATIVE PATHWAY | ABNORMALITIES | GLOMERULONEPHRITIS TYPE-II | UROLOGY & NEPHROLOGY | MONOCLONAL GAMMOPATHY | MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS | Glomerulonephritis, Membranoproliferative - pathology | Kidney - pathology | Complement C3 - metabolism | Humans | Middle Aged | Male | Treatment Outcome | Glomerulonephritis, Membranoproliferative - surgery | Kidney Transplantation | Paraproteinemias - complications | HLA Antigens - genetics | Young Adult | Glomerulonephritis, Membranoproliferative - immunology | Biopsy | Adolescent | Adult | Female | Child | Glomerulonephritis, Membranoproliferative - metabolism | Clinical Research
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7. Full Text New approaches to the treatment of dense deposit disease
by Smith, R.J.H and Alexander, J and Barlow, P.N and Botto, M and Cassavant, T.L and Cook, H.T and Cordoba, S.R. de and Hageman, G.S and Jokiranta, T.S and Kimberling, W.J and Lambris, J.D and Lanning, L.D and Levidiotis, V and Licht, C and Lutz, H.U and Meri, S and Pickering, M.C and Quigg, R.J and Rops, L.W.M.M and Salant, D.J and Sethi, S and Thurman, J.M and Tully, H.F and Tully, S.P and Vlag, J. van der and Walker, P.D and Wurzner, R and Zipfel, P.F and Dense Deposit Dis Focus Grp and Dense Deposit Disease Focus Group
Journal of the American Society of Nephrology, ISSN 1046-6673, 2007, Volume 18, Issue 9, pp. 2447 - 2456
The development of clinical treatment protocols usually relies on evidence-based guidelines that focus on randomized, controlled trials. For rare renal... 
COMPLEMENT FACTOR-H | HEMOLYTIC-UREMIC SYNDROME | MESANGIOCAPILLARY GLOMERULONEPHRITIS | RARE DISEASES | GLOMERULONEPHRITIS TYPE-II | UROLOGY & NEPHROLOGY | MACULAR DEGENERATION | RENAL-TRANSPLANTATION | IDIOPATHIC MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS | REDUCES PROTEINURIA | ALTERNATE-DAY PREDNISONE | Glomerulonephritis, Membranoproliferative - genetics | Animals | Glomerulonephritis, Membranoproliferative - physiopathology | Kidney - pathology | Humans | Glomerulonephritis, Membranoproliferative - diagnosis | Complement Pathway, Alternative | Glomerulonephritis, Membranoproliferative - therapy | Disease Progression | Kidney - physiopathology | Disease Models, Animal
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8. Full Text C3 Glomerulopathy: Clinicopathologic Features and Predictors of Outcome
by Nicholas R. Medjeral-Thomas and Michelle M. O’Shaughnessy and John A. O’Regan and Carol Traynor and Michael Flanagan and Limy Wong and Chia Wei Teoh and Atif Awan and Mary Waldron and Tom Cairns and Patrick O’Kelly and Anthony M. Dorman and Matthew C. Pickering and Peter J. Conlon and H. Terence Cook
Clinical Journal of the American Society of Nephrology, ISSN 1555-9041, 01/2014, Volume 9, Issue 1, pp. 46 - 53
Background and objectivesThe term C3 glomerulopathy describes renal disorders characterized by the presence of glomerular deposits composed of C3 in the... 
GLOMERULONEPHRITIS | ECULIZUMAB | COMPLEMENT FACTOR-H | ABNORMALITIES | MUTATION | UROLOGY & NEPHROLOGY | RISK | PATIENT | PROTEIN 5 | DEFICIENCY | DENSE-DEPOSIT DISEASE | Complement C3 - analysis | Prognosis | Kidney - pathology | Humans | Middle Aged | Kidney - immunology | Glomerulonephritis - diagnosis | Male | Glomerulonephritis, Membranoproliferative - complications | Young Adult | Time Factors | Ireland | Adult | Female | Retrospective Studies | Child | Glomerulonephritis, Membranoproliferative - pathology | Risk Factors | Biomarkers - analysis | Kaplan-Meier Estimate | Proportional Hazards Models | Glomerulonephritis - immunology | Glomerulonephritis - mortality | Glomerulonephritis - complications | Disease Progression | Glomerulonephritis, Membranoproliferative - immunology | Biopsy | Glomerulonephritis, Membranoproliferative - mortality | Glomerulonephritis - pathology | Adolescent | London | Glomerulonephritis, Membranoproliferative - diagnosis | Kidney Failure, Chronic - etiology | Original
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9. Full Text Causes of Alternative Pathway Dysregulation in Dense Deposit Disease
by Yuzhou Zhang and Nicole C. Meyer and Kai Wang and Carla Nishimura and Kathy Frees and Michael Jones and Louis M. Katz and Sanjeev Sethi and Richard J.H. Smith
Clinical Journal of the American Society of Nephrology, ISSN 1555-9041, 02/2012, Volume 7, Issue 2, pp. 265 - 274
Background and objectives This study was designed to investigate the causes of alternative pathway dysregulation in a cohort of patients with dense deposit... 
C-3 NEPHRITIC FACTOR | HYPOCOMPLEMENTEMIA | ACTIVATION | HEMOLYTIC-UREMIC SYNDROME | GLOMERULONEPHRITIS TYPE-II | UROLOGY & NEPHROLOGY | FACTOR-H | SERUM | MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS | AUTOANTIBODY | COMPLEMENT PATHWAY | Prognosis | Kidney - pathology | Autoantibodies - blood | Humans | Middle Aged | Kidney - immunology | Male | Glomerulonephritis, Membranoproliferative - blood | Mutation, Missense | Young Adult | Properdin - immunology | Time Factors | DNA Mutational Analysis | Adult | Female | Child | Erythrocytes - immunology | Complement Factor H - immunology | Enzyme-Linked Immunosorbent Assay | Kaplan-Meier Estimate | Genotype | Complement C3 Nephritic Factor - analysis | Biomarkers - blood | Complement C3-C5 Convertases - immunology | Glomerulonephritis, Membranoproliferative - genetics | Glomerulonephritis, Membranoproliferative - immunology | Iowa | Phenotype | Animals | Biopsy | Adolescent | Complement Factor B - immunology | Sheep | Glomerulonephritis, Membranoproliferative - diagnosis | Complement Factor H - genetics | Complement Pathway, Alternative | Immunoelectrophoresis, Two-Dimensional | Original
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10. Full Text Membranoproliferative Glomerulonephritis — A New Look at an Old Entity
by Sethi, Sanjeev and Fervenza, Fernando C
The New England Journal of Medicine, ISSN 0028-4793, 03/2012, Volume 366, Issue 12, pp. 1119 - 1131
This review discusses the causes, pathogenesis, and clinical management of membranoproliferative glomerulonephritis, which accounts for 7 to 10% of... 
FACTOR-H DEFICIENCY | MEDICINE, GENERAL & INTERNAL | DENSE DEPOSIT DISEASE | HEMOLYTIC-UREMIC SYNDROME | PRIMARY SJOGRENS-SYNDROME | MESANGIOCAPILLARY GLOMERULONEPHRITIS | ISOLATED C3 DEPOSITS | MONOCLONAL GAMMOPATHY | PRIMARY GLOMERULAR-DISEASES | ALTERNATE-DAY PREDNISONE | MYCOPHENOLATE-MOFETIL | Recurrence | Glucocorticoids - therapeutic use | Kidney - pathology | Humans | Kidney - immunology | Complement System Proteins - immunology | Kidney Transplantation | Glomerulonephritis, Membranoproliferative - therapy | Kidney - ultrastructure | Complement System Proteins - metabolism | Glomerulonephritis, Membranoproliferative - immunology | Complement Pathway, Alternative - physiology | Complement System Proteins - genetics | Glomerulonephritis, Membranoproliferative - diagnosis | Antigen-Antibody Complex - metabolism | Mutation | Glomerulonephritis, Membranoproliferative - etiology | Glomerulonephritis | Immunoglobulins | Bacterial infections | Transplants & implants | Disease | Pathogenesis | Infections | Genotype & phenotype | Hepatitis | Microscopy | Biopsy | Lymphomas | Autoimmune diseases | Viral infections
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11. Full Text Membranoproliferative glomerulonephritis with masked monotypic immunoglobulin deposits
by Larsen, Christopher P and Messias, Nidia C and Walker, Patrick D and Fidler, Mary E and Cornell, Lynn D and Hernandez, Loren H and Alexander, Mariam P and Sethi, Sanjeev and Nasr, Samih H
Kidney International, ISSN 0085-2538, 10/2015, Volume 88, Issue 4, pp. 867 - 873
The diagnosis of membranoproliferative glomerulonephritis (MPGN) has recently undergone change from an electron microscopy-based classification scheme to one... 
membranoproliferative glomerulonephritis | monoclonal gammopathy of renal significance | monoclonal gammopathy of undetermined significance | masked deposits | monoclonal gammopathy | C3 glomerulonephritis | IMMUNOFLUORESCENCE | DISEASE | RENAL SIGNIFICANCE | UROLOGY & NEPHROLOGY | SALVAGE TECHNIQUE | Complement C3 - analysis | Predictive Value of Tests | Humans | Middle Aged | Kidney Glomerulus - physiopathology | Male | Glomerulonephritis, Membranoproliferative - blood | False Positive Reactions | Paraffin Embedding | Kidney Glomerulus - ultrastructure | Diagnostic Errors | Female | Tissue Fixation | Databases, Factual | Glomerular Filtration Rate | Risk Factors | Biomarkers - analysis | Immunoglobulins - analysis | Microscopy, Electron | Glomerulonephritis, Membranoproliferative - immunology | Glomerulonephritis, Membranoproliferative - physiopathology | Biopsy | Fluorescent Antibody Technique | Creatinine - blood | Aged | Glomerulonephritis, Membranoproliferative - diagnosis | Kidney Glomerulus - immunology | Clinical Investigation
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12. Full Text Eculizumab for Dense Deposit Disease and C3 Glomerulonephritis
by Andrew S. Bomback and Richard J. Smith and Gaetano R. Barile and Yuzhou Zhang and Eliot C. Heher and Leal Herlitz and M. Barry Stokes and Glen S. Markowitz and Vivette D. D’Agati and Pietro A. Canetta and Jai Radhakrishnan and Gerald B. Appel
Clinical Journal of the American Society of Nephrology, ISSN 1555-9041, 05/2012, Volume 7, Issue 5, pp. 748 - 756
Background and objectives The principle defect in dense deposit disease and C3 glomerulonephritis is hyperactivity of the alternative complement pathway.... 
COMPLEMENT | GLOMERULOPATHY | HEMOLYTIC-UREMIC SYNDROME | ALTERNATIVE PATHWAY | MUTATION | UROLOGY & NEPHROLOGY | MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS | Antibodies, Monoclonal, Humanized - adverse effects | Glomerulonephritis, Membranoproliferative - pathology | Antibodies, Monoclonal, Humanized - therapeutic use | Complement Membrane Attack Complex - metabolism | Humans | Complement Factor I - genetics | Male | Glomerulonephritis, Membranoproliferative - drug therapy | Young Adult | Glomerulonephritis, Membranoproliferative - genetics | Complement Factor B - genetics | Proteinuria - urine | Creatinine - blood | Adult | Complement System Proteins - genetics | Membrane Cofactor Protein - genetics | Complement Factor H - genetics | Complement C5 - antagonists & inhibitors | Proteinuria - etiology | Original
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13. Full Text Membranoproliferative Glomerulonephritis: Pathogenetic Heterogeneity and Proposal for a New Classification
by Sethi, Sanjeev, MD, PhD and Fervenza, Fernando C., MD, PhD
Seminars in Nephrology, ISSN 0270-9295, 2011, Volume 31, Issue 4, pp. 341 - 348
Summary Membranoproliferative glomerulonephritis (MPGN) is a pattern of injury that results from subendothelial and mesangial deposition of Igs caused by... 
Nephrology | Membranoproliferative glomerulonephritis | monoclonal gammopathy | C3 glomerulopathy | alternative pathway of complement | MGUS | C3-GN | MPGN | Monoclonal gammopathy | Alternative pathway of complement | LONG-TERM | PRIMARY SJOGRENS-SYNDROME | MESANGIOCAPILLARY GLOMERULONEPHRITIS | BACTERIAL-ENDOCARDITIS | DENSE DEPOSIT DISEASE | KIDNEY-TRANSPLANTATION | PROLIFERATIVE GLOMERULONEPHRITIS | UNDETERMINED SIGNIFICANCE | UROLOGY & NEPHROLOGY | TRANSPLANT GLOMERULOPATHY | Glomerulonephritis, Membranoproliferative - pathology | Glomerulonephritis, Membranoproliferative - immunology | Glomerulonephritis, Membranoproliferative - classification | Complement C3 - metabolism | Humans | Complement Pathway, Alternative | Glomerulonephritis, Membranoproliferative - etiology
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