Author:
Watts Andrew J.B.,Keller Keith H.,Lerner Gabriel,Rosales Ivy,Collins A. Bernard,Sekulic Miroslav,Waikar Sushrut S.,Chandraker Anil,Riella Leonardo V.,Alexander Mariam P.,Troost Jonathan P.,Chen Junbo,Fermin Damian,Yee Jennifer Lai,Sampson Matthew,Beck Laurence H.,Henderson Joel M.,Greka Anna,Rennke Helmut G.,Weins Astrid
Abstract
AbstractDysfunction of podocytes, cells critical for glomerular filtration, underlies proteinuria and kidney failure. Genetic forms of proteinuric kidney disease can be caused by mutations in several podocyte genes, including nephrin, a critical component of the kidney filter. In contrast, the etiology of acquired acute-onset nephrotic syndrome has remained elusive. Here we identify autoantibodies against nephrin in serum and glomeruli of a subset of adults and children with non-congenital acute nephrotic syndrome. Our findings align with published experimental animal studies and elucidate a novel autoimmune phenomenon in proteinuric kidney disease interfering with glomerular filter integrity.
Publisher
Cold Spring Harbor Laboratory