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

The renal disorder C3 glomerulopathy with dense deposit disease (C3G-DDD) pattern results from complement dysfunction and primarily affects children and young adults. There is no effective treatment, and patients often progress to end-stage renal failure. A small fraction of C3G-DDD cases linked to...

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Veröffentlicht in:	The Journal of clinical investigation 2014-01, Vol.124 (1), p.145-155
Hauptverfasser:	Chen, Qian, Wiesener, Michael, Eberhardt, Hannes U, Hartmann, Andrea, Uzonyi, Barbara, Kirschfink, Michael, Amann, Kerstin, Buettner, Maike, Goodship, Tim, Hugo, Christian, Skerka, Christine, Zipfel, Peter F
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult Base Sequence Biology Biomedical research Biopsy Care and treatment Child, Preschool Chromosome Deletion Complement Activation Complement C3-C5 Convertases - metabolism Complement C3b - metabolism Complement C3b Inactivator Proteins - genetics Complement C3b Inactivator Proteins - metabolism Complement System Proteins - genetics Complement System Proteins - metabolism Development and progression DNA Mutational Analysis Enzyme Stability Genes Genetic aspects Genetic variation Glomerulonephritis Glomerulonephritis, Membranoproliferative - blood Glomerulonephritis, Membranoproliferative - genetics Glomerulonephritis, Membranoproliferative - therapy HEK293 Cells Histology Humans Infections Kidney - pathology Kidney diseases Kidney Failure, Chronic - blood Kidney Failure, Chronic - genetics Kidney Failure, Chronic - therapy Male Microscopy Mutant Chimeric Proteins - genetics Mutant Chimeric Proteins - metabolism Natural products Patients Physiological aspects Plasma Plasmapheresis Protein Binding Proteins Sequence Deletion Studies Treatment Outcome Young Adult
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