대한신장학회

Skip Navigation: Skip to contents

KOR
ENG
전체메뉴보기

간행물 검색

현재 페이지 경로

HOME
간행물
간행물 검색

논문분류	춘계학술대회 초록집
제목	Nephrin Autoantibodies in Idiopathic Nephrotic Syndrome: Mechanism, Marker, Clinical Implications
저자	Tobias B. Huber
출판정보	2024; 2024(1):
키워드
초록	Minimal change disease (MCD) and primary focal segmental glomerulosclerosis (FSGS) in adults, along with idiopathic nephrotic syndrome (INS) in children, are immune-mediated podocytopathies leading to nephrotic syndrome. Autoantibodies targeting nephrin have been identified in MCD patients, but their roles are unclear. In a multicenter study analyzing antinephrin autoantibodies in adults with various glomerular diseases, children with INS, and controls, a total of 539 patients (357 adults, 182 children) and 117 controls were included. Among adults, antinephrin autoantibodies were found in 46 of 105 (44%) with MCD, 7 of 74 (9%) with primary FSGS, and rarely in other conditions. In children with INS, 94 of 182 (52%) had detectable antinephrin autoantibodies. In untreated patients with active MCD or INS, prevalence was 69% and 90%, respectively. Antinephrin autoantibody levels correlated with disease activity. Experimental immunization in mice induced nephrotic syndrome, an MCD-like phenotype, IgG localization to the podocyte slit diaphragm, nephrin phosphorylation, and severe cytoskeletal changes. This study indicates that circulating antinephrin autoantibodies are common in MCD and INS, serving as markers of disease activity. Their binding at the slit diaphragm induces podocyte dysfunction and nephrotic syndrome, underscoring their pathophysiological significance.
원문(PDF)	PDF 원문보기

목록

위로가기