Dimerix (ASX:DXB) has found itself at the centre of a significant global rare-disease transaction, following BioMarin Pharmaceutical’s US$4.8 billion acquisition of Amicus Therapeutics, the Australian company’s U.S. commercial licensing partner.
The deal transfers Amicus’s rights and obligations under its licensing agreement with Dimerix to BioMarin, while leaving the underlying agreement intact and in force.
Dimerix said the transaction validates the commercial potential of its lead Phase 3 asset, DMX-200, which is being developed for focal segmental glomerulosclerosis (FSGS). FSGS is a rare, severe kidney disease with no approved targeted therapies in the United States.
Under the existing U.S. licensing agreement, Dimerix remains eligible to receive up to US$590 million in upfront, development, and sales milestone payments, in addition to tiered royalties in the low teens to low twenties on net sales. To date, the company has already received US$30 million upon execution of the agreement in May 2025.
BioMarin is a global rare-disease specialist with deep experience in regulatory approvals, reimbursement strategy, manufacturing and commercialisation. Dimerix said the commercial licensing agreement will remain unchanged until the transaction is completed, with Amicus and Dimerix continuing to collaborate on U.S. planning in the interim.
The deal does not affect Dimerix’s licensing arrangements outside the United States. Separate agreements with Advanz Pharma, Taiba, and Fuso remain in place, collectively providing up to approximately AU$1.4 billion in potential upfront and milestone payments, plus royalties. Across all territories, Dimerix has now received more than AU$65 million in payments.
At the same time, Dimerix continues to advance the ACTION3 Phase 3 trial, a pivotal, multi-centre, randomised, double-blind study evaluating DMX-200 in patients with FSGS who are receiving standard angiotensin II receptor blocker therapy. The trial is designed to generate definitive evidence of proteinuria reduction and preservation of kidney function over a two-year treatment period, supporting a potential marketing approval application.
FSGS remains an area of profound unmet need. More than 40,000 people in the United States are estimated to be living with the disease, which can progress rapidly to end-stage kidney failure. Even among patients who receive kidney transplants, recurrence rates can reach 60%, underscoring the urgent need for disease-modifying treatments.
Dimerix Chief Executive Officer Dr Nina Webster said the BioMarin transaction highlights growing commercial and strategic interest in rare kidney diseases. She described BioMarin as a patient-focused global company with a strong track record in rare-disease development and commercialisation, and said the deal is “fantastic news for patients with rare diseases, including FSGS, who currently have a poor prognosis and very limited treatment options.”