A recent study published by Sethi et al. demonstrated the presence of two proteins, Exostosin1 (EXT1) and Exostosin 2 (EXT2), along the GBM of PLA2R-negative MN. In this study, the authors showed that 81% of the EXT1/EXT2-associated MN showed features of autoimmune disease, including membranous lupus nephritis.
Sethi S, et al. Exostosin1/Exostosin 2-Associated Membranous Nephropathy. JASN 2019; 30:1123-1136.
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