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    Mitochondrial Myopathies Companies

    ID: MRFR/Pharma/2452-HCR
    110 Pages
    Rahul Gotadki
    October 2025

    Mitochondrial myopathies are a group of neuromuscular disorders caused by dysfunctional mitochondria, the energy-producing structures within cells. Companies involved in mitochondrial myopathies may focus on developing diagnostic tools, treatment options, and therapies to address the underlying mitochondrial dysfunction.

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    Top Industry Leaders in the Mitochondrial Myopathies Market

    Mitochondrial Myopathies Key CompaniesLatest Mitochondrial Myopathies Companies Update



    • Jul 2023: Reata Pharmaceuticals, Inc. and Biogen Inc. announced that they have reached a definitive agreement whereby Biogen will purchase Reata for cash for $172.50 per share, or an estimated $7.3 billion. Reata has made great strides in developing treatments that control inflammation and cellular metabolism in severe neurological disorders. With a commercial launch pending and an ongoing regulatory evaluation in Europe, Reata's FDA-approved SKYCLARYS® (omaveloxolone) is the initial and only licensed medication for Friedreich's ataxia (FA) in the United States. Reata is also creating a portfolio of cutting-edge treatments for various neurological conditions.




    • Mar 2023: Mavodelpar (REN001), a strong and selective PPARδ agonist, is being tested in the Phase IIb STRIDE study by Reneo Pharmaceuticals to treat primary mitochondrial myopathies (PMM). The company has successfully reached its goal of enrollment in this trial. The purpose of the double-masked, randomized, placebo-controlled, multinational pivotal trial is to assess mavodelpar in adult patients with PMM caused by abnormalities in the mitochondria (mtDNA). Over 24 weeks, mavodelpar, given orally at 100 mg once a day, will be evaluated for safety and effectiveness. The primary effectiveness objective of the trial is the difference from baseline in the amount of distance walked during the 12-minute walk test at week 24.


    List of Mitochondrial Myopathies Key companies in the market

    • Reata Pharmaceuticals, Inc.

    • Stealth BioTherapeutics

    • Raptor Pharmaceutical Corp.

    • GeneDx

    • NeuroVive Pharmaceutical AB