Factor H-Related Protein 1 Drives Disease Susceptibility and Prognosis in C3 Glomerulopathy.
Bárbara Márquez-TiradoJosué Gutiérrez-TenorioAgustín TortajadaLaura Lucientes ContinenteFernando Caravaca-FontánTalat H MalikRaquel Roldán MonteroSandra ElíasAna Saiz GonzalezGema Fernández-JuarezPilar Sánchez-CorralMatthew Caleb PickeringManuel PragaSantiago Rodríguez de CórdobaElena Goicoechea de JorgePublished in: Journal of the American Society of Nephrology : JASN (2022)
Our findings explain how FHR-1 mutants with duplicated dimerization domains result in predisposition to C3G. They also provide a pathogenic mechanism that may be shared by other diseases, such as IgA nephropathy or age-related macular degeneration, and identify FHR-1 as a potential novel therapeutic target in C3G.