ASN's Mission

To create a world without kidney diseases, the ASN Alliance for Kidney Health elevates care by educating and informing, driving breakthroughs and innovation, and advocating for policies that create transformative changes in kidney medicine throughout the world.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on X

Kidney Week

Abstract: TH-PO0659

Therapeutic Targeting of CLDN1 Reduces Glomerular Crescent Formation and Fibrosis and Preserves Renal Function in ANCA-Associated Glomerulonephritis

Session Information

Category: Glomerular Diseases

  • 1401 Glomerular Diseases: Mechanisms, including Podocyte Biology

Authors

  • Teixeira, Geoffrey, Alentis Therapeutics AG, Allschwil, BL, Switzerland
  • Dang, Minh Huan, Monash University, Melbourne, Victoria, Australia
  • Prakongtham, Peemapat, Monash University, Melbourne, Victoria, Australia
  • Lu, Chunni, Monash University, Melbourne, Victoria, Australia
  • Polini, Elisa, Alentis Therapeutics AG, Allschwil, BL, Switzerland
  • Chanemouga, Isabelle, Alentis Therapeutics AG, Allschwil, BL, Switzerland
  • Cavadini, Silvia, Alentis Therapeutics AG, Allschwil, BL, Switzerland
  • Anquetil, Vincent, Alentis Therapeutics AG, Allschwil, BL, Switzerland
  • Herbert, Corentin, Alentis Therapeutics AG, Allschwil, BL, Switzerland
  • Iacone, Roberto, Alentis Therapeutics AG, Allschwil, BL, Switzerland
  • Kitching, A. Richard, Monash University, Melbourne, Victoria, Australia
Background

Despite immunosuppression, glomerular crescents in anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis (crGN) can progress to fibrosis and glomerulosclerosis, underscoring the need for adjunctive therapies targeting fibrotic pathways. In crGN, claudin-1 (CLDN1), a transmembrane tight junction protein, becomes aberrantly exposed outside tight junctions, where it promotes pro-fibrotic signaling, immune cell homing and extracellular matrix (ECM) remodeling. Lixudebart (ALE.F02), a first-in-class monoclonal antibody (mAb) developed by Alentis, selectively targets and blocks exposed CLDN1 on activated renal epithelial cells. A Phase II clinical trial is currently underway in patients with crGN due to AAV (NCT06047171). This study investigates whether CLDN1 inhibition modulates established ANCA-induced crGN.

Methods

A new 21 day model of crGN in mice induced by ANCA (mouse anti-mouse myeloperoxidase mAbs) tested anti-CLDN1 mAb therapy, commenced 6 days post disease induction (d6 crescents 31.6±3.7%). Mechanistic studies used in vitro/ex vivo epithelial cell models to explore interactions between CLDN1 and pro-inflammatory/pro-fibrotic mediators.

Results

In murine MPO-AAV, anti-CLDN1 mAb treatment reduced glomerular crescents (d21 Ctrl 31.4±2.6%, anti-CLDN1 mAb 19.1±1.7% p<0.001), limited glomerular segmental necrosis, decreased glomerular fibrosis (d21 Ctrl 12.5±0.6%, anti-CLDN1 mAb 9.7±0.7% p=0.0210) and improved renal function. Ex vivo, Lixudebart disrupted CLDN1 interactions with CD44 and MMP14, key mediators of immune cell recruitment and ECM remodeling.

Conclusion

Lixudebart commenced in established experimental MPO-ANCA induced crGN improved histology and kidney function. In vitro/ex vivo studies link CLDN1’s aberrant exposure to immune cell recruitment and ECM remodeling. These studies support CLDN1 inhibition as a novel therapeutic strategy in crGN.

Digital Object Identifier (DOI)