Elevated factor H–related protein 1 and factor H pathogenic variants decrease complement regulation in IgA nephropathy

IgA nephropathy (IgAN), a frequent cause of chronic kidney disease worldwide, is characterized by mesangial deposition of galactose-deficient IgA1-containing immune complexes. Complement involvement in IgAN pathogenesis is suggested by the glomerular deposition of complement components and the stron...

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Veröffentlicht in:	Kidney international 2017-10, Vol.92 (4), p.953-963
Hauptverfasser:	Tortajada, Agustín, Gutiérrez, Eduardo, Goicoechea de Jorge, Elena, Anter, Jaouad, Segarra, Alfons, Espinosa, Mario, Blasco, Miquel, Roman, Elena, Marco, Helena, Quintana, Luis F., Gutiérrez, Josué, Pinto, Sheila, Lopez-Trascasa, Margarita, Praga, Manuel, Rodriguez de Córdoba, Santiago
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult Blood Proteins - genetics CFH mutation Cohort Studies complement alternative pathway Complement C3b Inactivator Proteins - analysis Complement C3b Inactivator Proteins - genetics Complement Factor H - analysis Complement Factor H - genetics Complement Pathway, Alternative - genetics Disease Progression factor H factor H-related proteins Female Glomerular Filtration Rate Glomerulonephritis, IGA - blood Glomerulonephritis, IGA - genetics Humans IgAN Male Middle Aged Polycystic Kidney, Autosomal Dominant - blood Polycystic Kidney, Autosomal Dominant - genetics Renal Insufficiency, Chronic - blood Renal Insufficiency, Chronic - genetics Young Adult
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