Japan approves Amicus Therapeutics’ treatment for late-onset Pompe disease

Published 25/06/2025, 12:18
Japan approves Amicus Therapeutics’ treatment for late-onset Pompe disease

TOKYO/PRINCETON - Japan’s Ministry of Health, Labour and Welfare has approved Pombiliti (cipaglucosidase alfa) + Opfolda (miglustat) for the treatment of adult patients with late-onset Pompe disease (LOPD), Amicus Therapeutics (NASDAQ:FOLD), a $1.76 billion market cap biotech company with impressive 90.6% gross profit margins, announced Wednesday.

The two-component therapy is designed to address LOPD, an inherited lysosomal disorder caused by deficiency of the enzyme acid alpha-glucosidase (GAA), which leads to progressive muscle weakness throughout the body.

Pombiliti is a recombinant human GAA enzyme designed for increased uptake into muscle cells, while Opfolda functions as an enzyme stabilizer in the blood.

The approval was based on clinical data from the Phase 3 PROPEL study, which included both enzyme replacement therapy (ERT)-naïve and ERT-experienced participants.

"We are delighted that we will now be able to offer a compelling new treatment option to patients living with late-onset Pompe disease in Japan," said Bradley Campbell, President and Chief Executive Officer of Amicus Therapeutics.

With this regulatory decision, the therapy is now approved in the United States, European Union, United Kingdom, Canada, Australia, Switzerland, and Japan.

The treatment carries warnings for potential hypersensitivity reactions including anaphylaxis, infusion-associated reactions, and risk of acute cardiorespiratory failure in susceptible patients. Common adverse reactions include headache, diarrhea, fatigue, nausea, abdominal pain, and pyrexia.

The information in this article is based on a press release statement from Amicus Therapeutics.

In other recent news, Amicus Therapeutics reported first-quarter 2025 earnings that surpassed analyst expectations, with adjusted earnings per share at $0.03 compared to the anticipated -$0.02. However, the company’s revenue of $125.25 million fell short of Wall Street’s forecast of $136.65 million. Despite the revenue miss, Amicus saw a 15% year-over-year revenue growth, with Galafold sales increasing by 6% to $104.2 million and Pombiliti + Opfolda sales jumping 92% to $21.0 million. The company adjusted its total revenue growth guidance for 2025 to 15-22% at constant exchange rates, down from the previous 17-24% outlook. Amicus maintained its Galafold revenue growth guidance but lowered expectations for Pombiliti + Opfolda. The company reiterated its aim to achieve GAAP profitability in the second half of 2025 and ended the quarter with $250.6 million in cash and marketable securities. Additionally, Amicus announced it had licensed U.S. commercial rights to Dimerix’s DMX-200, a Phase 3 treatment for a rare kidney disease.

This article was generated with the support of AI and reviewed by an editor. For more information see our T&C.

Latest comments

Risk Disclosure: Trading in financial instruments and/or cryptocurrencies involves high risks including the risk of losing some, or all, of your investment amount, and may not be suitable for all investors. Prices of cryptocurrencies are extremely volatile and may be affected by external factors such as financial, regulatory or political events. Trading on margin increases the financial risks.
Before deciding to trade in financial instrument or cryptocurrencies you should be fully informed of the risks and costs associated with trading the financial markets, carefully consider your investment objectives, level of experience, and risk appetite, and seek professional advice where needed.
Fusion Media would like to remind you that the data contained in this website is not necessarily real-time nor accurate. The data and prices on the website are not necessarily provided by any market or exchange, but may be provided by market makers, and so prices may not be accurate and may differ from the actual price at any given market, meaning prices are indicative and not appropriate for trading purposes. Fusion Media and any provider of the data contained in this website will not accept liability for any loss or damage as a result of your trading, or your reliance on the information contained within this website.
It is prohibited to use, store, reproduce, display, modify, transmit or distribute the data contained in this website without the explicit prior written permission of Fusion Media and/or the data provider. All intellectual property rights are reserved by the providers and/or the exchange providing the data contained in this website.
Fusion Media may be compensated by the advertisers that appear on the website, based on your interaction with the advertisements or advertisers
© 2007-2025 - Fusion Media Limited. All Rights Reserved.