Generation of Human Cardiomyocytes With Adult-Like Metabolism by a Chemically Defined Medium for Mitochondrial Disease Modeling.

MM11, an 11-component chemically defined medium, drives adult-like mitochondrial maturation in human iPSC cardiomyocytes, including interfibrillar mitochondria and respiratory supercomplexes.

• Why it matters: iPSC cardiomyocytes are metabolically immature, limiting faithful modeling of mitochondria-dependent diseases like Friedreich ataxia-associated cardiomyopathy and other cardiac pathologies.
• What they did: iPSC cardiomyocytes were treated with MM11 for 2 weeks using molecular, structural, and functional assays, benchmarked against isolated primary adult human cardiomyocytes, including oxygen consumption and 13C isotope tracing.
• The result: MM11 increased mitochondrial respiration and fatty acid metabolism and enabled fatty-acid–linked oxidative phosphorylation, improving modeling of Friedreich ataxia cardiomyopathy and ischemia-reperfusion injury.

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