Related Experiment Video
Updated: Jun 25, 2025
Isolation of Glomeruli and In Vivo Labeling of Glomerular Cell Surface Proteins
Published on: January 18, 2019
Autoantibodies Targeting Nephrin in Podocytopathies
Felicitas E Hengel1, Silke Dehde1, Moritz Lassé1
1From the III. Department of Medicine (F.E.H., S.D., M.L., G.Z., L.S., A.S., O.K., F.G., R.L., L.M.M., E.H., M.M.R., N.M.T., T.B.H.) and the Departments of Medical Biometry and Epidemiology (H.O.P.), Pathology (T.Z., T.W.), Pediatric Nephrology (J.O.), and Obstetrics and Fetal Medicine (A.D.), Hamburg Center for Kidney Health (F.E.H., S.D., M.L., G.Z., L.S., A.S., O.K., F.G., R.L., L.M.M., J.O., E.H., M.M.R., N.M.T., T.B.H.), and the Hamburg Center for Translational Immunology (N.M.T., T.B.H.), University Medical Center Hamburg-Eppendorf, Hamburg, and the Center for Pediatrics and Adolescent Medicine, University Hospital Heidelberg, Heidelberg (F.S.) - all in Germany; the Department of Biomedicine, Aarhus University, Aarhus, Denmark (F.D., A.M.B., M.M.R.); the Department of Precision and Regenerative Medicine and Ionian Area, Nephrology, Dialysis, and Transplantation Unit, University of Bari Aldo Moro, Bari (A.M., P.P., L.G.), and the Division of Nephrology, Bambino Gesù Children's Hospital, IRCCS, Rome (M.C., F.E., M.V.) - both in Italy; INSERM, Unité Mixte de Recherche S 1155, Sorbonne Université (H.D., P.R.), and the Pediatric Nephrology Department, Robert Debré Hospital, Assistance Publique-Hôpitaux de Paris (C.D., J.H.), Paris, and the Division of Nephrology, Centre Hospitalier du Mans, Le Mans (P.R.) - all in France; the Center for Clinical and Translational Research, Abigail Wexner Research Institute at Nationwide Children's Hospital, and the Department of Pediatrics, Ohio State University College of Medicine - both in Columbus (W.E.S.); the Department of Pathology, Brigham and Women's Hospital, and Harvard Medical School - both in Boston (A.W.); and the Department of Medicine, Johns Hopkins University School of Medicine, Baltimore (N.A.).
Autoantibodies targeting nephrin are common in minimal change disease and idiopathic nephrotic syndrome, acting as markers of disease activity and causing nephrotic syndrome by damaging podocytes.
Area of Science:
- Nephrology
- Immunology
- Pathophysiology
Background:
- Minimal change disease (MCD) and idiopathic nephrotic syndrome (INS) are immune-mediated podocytopathies causing nephrotic syndrome.
- The role of autoantibodies against nephrin in these conditions was previously unclear.
Purpose of the Study:
- To investigate the prevalence and role of antinephrin autoantibodies in adult and pediatric nephrotic syndromes.
- To establish a link between antinephrin autoantibodies and disease activity and pathogenesis.
Main Methods:
- A multicenter study analyzed antinephrin autoantibodies in 539 patients with various glomerular diseases and 117 controls.
- An experimental mouse model was developed via active immunization with recombinant murine nephrin.
Main Results:
- Antinephrin autoantibodies were detected in 44% of adult MCD patients and 52% of pediatric INS patients.
- Antibody levels correlated with disease activity, and experimental immunization in mice induced nephrotic syndrome and podocyte damage.
Conclusions:
- Circulating antinephrin autoantibodies are prevalent in MCD and INS and serve as markers of disease activity.
- These autoantibodies contribute to podocyte dysfunction and nephrotic syndrome pathogenesis through slit diaphragm binding.
Related Concept Videos
01:10Nephrons
01:20Renal Corpuscle
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...