Avalglucosidase alfa

Shionogi CEO Isao Teshirogi, Ph.D., said Maze Therapeutics’ MZE001 is a “strong strategic fit” for his company. It appears Sanofi’s loss is Shionogi’s gain. Five months after the Federal Trade Commission (FTC) scuttled Sanofi’s attempt to acquire Maze Therapeutics’ Pompe disease program, Japan’s Shionogi has scooped up the asset. Maze and Sanofi unveiled their own licensing deal for the program, MZE001, back in May 2023. The French pharma agreed to pay $150 million upfront along with the potential for $605 million in milestones for worldwide rights to a drug candidate that could treat Pompe by stopping the buildup of glycogen. It looked like a good fit for Sanofi’s pipeline, where MZE001 would have sat alongside another Pompe treatment in the form of enzyme replacement therapy Nexviazyme. But the FTC saw that as a problem, arguing that the deal would “eliminate a nascent competitor poised to challenge Sanofi’s monopoly in the Pompe disease therapy market.” While Sanofi objected to the move, it didn't formally contest the agency's decision. By December 2023, the drugmaker had announced it was terminating the agreement. Step forward, Shionogi, which revealed this morning that it has acquired MZE001 for itself for the same upfront price tag of $150 million. While milestone payments are also attached to the deal, the precise amount wasn’t disclosed in the May 10 release. The upfront fee is in a different league to the 300 million Japanese yen ($1.9 million) Shionogi paid for its previous licensing deal in March—an anti-IgE antibody-induced peptide for seasonal allergic rhinitis developed by fellow Japanese biotech FunPep. Shionogi CEO Isao Teshirogi, Ph.D., said Maze’s MZE001 is a “strong strategic fit” for his company. “It will help meaningfully advance our commitment to develop innovative medicines that address unmet medical needs and complement Shionogi's rapidly expanding pipeline in the focus areas designated in our medium-term business plan,” Teshirogi added.  Shionogi pointed to the drug’s potential to become the first oral treatment for Pompe disease. If approved, the GYS1 inhibitor could be used both as a monotherapy or as an add-on therapy for enzyme replacement, which is the current standard of care for the condition, the company added. Paul Hudson may be another person who’s pleased to see MZE001 find a home. The Sanofi CEO has previously expressed concern that the failure to get their own licensing deal over the line put the drug’s future at risk. “Our first instincts on Pompe were, we’re just disappointed, that there’s a risk the program doesn’t continue,” Hudson said at the J.P. Morgan Healthcare Conference in January. “I just think sometimes capital is hard to come by; I think sometimes expertise isn't there, and I think sometimes in these smaller companies their desire was to get it to this point.”

Maze Therapeutics looks to have found another partner for its investigational Pompe disease drug MZE001 after a deal last year with Sanofi fell foul of antitrust regulators. In an agreement announced on Friday, Maze will receive $150 million upfront from Shionogi and is eligible for additional milestones payments and tiered sales royalties.Sanofi abandoned its plans to license the Maze molecule in December under a deal potentially worth $750 million, including $150 million upfront, after the US Federal Trade Commission (FTC) raised competition concerns. The French drugmaker already markets the Pompe disease enzyme replacement therapies Lumizyme (alglucosidase alfa), also known as Myozyme, and Nexviazyme (avalglucosidase alfa), which are both administered via biweekly intravenous infusions.Meanwhile, Shionogi is looking to strengthen its rare diseases portfolio as part of its revised medium-term business strategy. “The science behind MZE001 is differentiated and promising,” with the potential to serve as “both mono and add-on therapy to enzyme replacement therapies,” remarked Shionogi CEO Isao Teshirogi. MZE001, an oral inhibitor of GYS1, is designed to restrict glycogen accumulation in patients with Pompe disease. The drug’s Phase I data released in February last year confirmed target engagement, prompting Maze to finalise mid-stage development. As part of the deal for MZE001, Shionogi will also have global rights to related programmes and intellectual property.