• Dr. Vincenzo L'Imperio

NELL-1: new antigen in MN

Aggiornato il: 26 ott 2019

Background

Since 2009, three different antigen have been associated to MN (PLA2R, THSD7A, EXT1/EXT2), with around 20-30% of the cases remaining "orphan" of a biomarker


Take home messages

  • NELL1 is the second most frequent antigen in MN (16% of the negative-MN cases)

  • Patients with NELL1-MN have circulating autoantibodies to the antigen, in one case decreasing after therapy with RTX

  • Autoantibodies recognize conformation dependent epitopes (such as in the case of PLA2R/THSD7A)

  • The incidence of NELL1-MN is different in the American (23%) and in the European (5.9%) cohorts, suggesting different genetic background as influencing factor

  • Tissue anti-NELL1 autoantibodies are more frequently of IgG1 subclass (as compared to the IgG4 dominant anti-PLA2R)

  • Five cases (all European) are linked with malignancies



https://www.kidney-international.org/article/S0085-2538(19)30995-0/abstract


#membranousnephropathy #renalpath #nephropath #MN #immunofluorescence #electronmicroscopy

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Sito della Scuola di Anatomia Patologica

dell'Università degli Studi di Milano-Bicocca

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