Primary membranous nephropathy (also known as idiopathic membranous nephropathy,
IMN) is an organ specific autoimmune kidney disease characterized by the development
of immune complex deposits in the sub-epithelial spaces, podocyte effacement and
glomerular capillary wall thickening in the later stages. Clinical studies have demonstrated
that over 70% of patients with IMN possess circulating autoimmune antibodies specifically
targeting the phospholipase A2 receptor (PLA2R) on the surface of podocytes. The autoantibodies
only bind to the extracellular portion of PLA2R under the non-reducing condition, indicating that the
epitope in PLA2R is conformational requiring specific disulfide bonds to maintain its structure. We recently
have successfully located the dominant epitope in PLA2R to the extreme N-terminus of the receptor. This
finding has opened a new direction for understanding the pathogenesis of anti-PLA2R autoantibody induced
IMN and offered a strong basis for developing sensitive clinical assays for IMN diagnosis and prognosis, and
potentially, new therapeutic approaches for IMN treatment.
Keywords: Apheresis column, enzyme-linked immunosorbent assay (ELISA), epitope, idiopathic membranous
nephropathy (IMN), immunotolerance therapy, integrin 31, kidney, mechanism, pathogenesis,
phospholipase A2 receptor (PLA2R).
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