Complement inhibition in C3 glomerulopathy

Highlights • Dysregulation of the alternative pathway of complement plays a critical role in C3 glomerulopathy. • Ultra-rare variants in complement genes are identified in about 40% of C3 glomerulopathy patients. • C3 nephritic factors are present in the majority of patients with C3 glomerulopathy....

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Veröffentlicht in:Seminars in immunology 2016-06, Vol.28 (3), p.241-249
Hauptverfasser: Nester, Carla M, Smith, Richard J.H
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description Highlights • Dysregulation of the alternative pathway of complement plays a critical role in C3 glomerulopathy. • Ultra-rare variants in complement genes are identified in about 40% of C3 glomerulopathy patients. • C3 nephritic factors are present in the majority of patients with C3 glomerulopathy. • Complement biomarker abnormalities are common in C3 glomerulopathy. • Complement inhibitors provide a targeted approach to the treatment of C3 glomerulopathy.
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subjects Allergy and Immunology
Animals
Biomarkers - metabolism
C3 glomerulonephritis
C3 glomerulopathy
Complement
Complement C3 - genetics
Complement C3 - immunology
Complement Inactivating Agents - therapeutic use
Complement Pathway, Alternative
Dense deposit disease
Eculizumab
Gene Frequency
Genotype
Glomerulonephritis, Membranous - genetics
Glomerulonephritis, Membranous - immunology
Glomerulonephritis, Membranous - therapy
Humans
Immunotherapy - methods
Kidney - metabolism
Kidney - pathology
Membranoproliferative glomerulonephritis
Polymorphism, Genetic
title Complement inhibition in C3 glomerulopathy
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