CSL and Alentis Therapeutics have entered into an exclusive global collaboration agreement to co-develop and co-promote lixudebart, an investigational monoclonal antibody targeting claudin-1 for potential use across kidney, liver and other diseases.

Lixudebart is currently being evaluated in the Phase 2 RENAL trial in patients with ANCA-associated vasculitis with rapidly progressive glomerulonephritis (AAV-RPGN), a rare and potentially life-threatening autoimmune disease that can cause rapid kidney function loss, irreversible kidney damage and end-stage renal disease. Under the collaboration, the companies will also advance lixudebart in Focal Segmental Glomerulosclerosis (FSGS), a progressive kidney disease, and Primary Sclerosing Cholangitis (PSC), a chronic autoimmune liver disease.

Lixudebart selectively targets exposed claudin-1, which is involved in inflammatory and fibrotic signalling pathways. The companies are evaluating its potential to deliver anti-inflammatory and anti-fibrotic effects and prevent or reverse organ damage across kidney, liver and lung diseases.

Under the agreement, CSL will make an initial payment of USD 1.2 billion in additional commercial milestone payments. CSL will fully fund completion of the ongoing Phase 2 RENAL trial and the planned Phase 3 trial in AAV-RPGN, as well as Phase 2 trials in FSGS and PSC and other supporting development activities. Following commercialisation, global profits will be shared 55% by CSL and 45 percent by Alentis.

An interim analysis of 26 patients with AAV-RPGN in the ongoing Phase 2 RENAL trial showed improvements in kidney function, assessed through estimated Glomerular Filtration Rate (eGFR) and proteinuria, at 24 weeks. In the Phase 1b FEGATO trial involving 41 patients with advanced F3/F4 liver fibrosis, lixudebart demonstrated improved liver function at six weeks. The studies also showed dose-dependent claudin-1 target engagement, with a favourable safety and tolerability profile.

Lixudebart, formerly known as ALE.F02, has also received Orphan Drug designation from the US Food and Drug Administration for the treatment of Idiopathic Pulmonary Fibrosis (IPF).

The collaboration combines Alentis’ expertise in claudin-1-targeted therapies with CSL’s clinical development and commercialisation capabilities in nephrology. Alentis is also developing oncology candidates ALE.P02 and ALE.P03, targeting claudin-1 for cancer indications.