Figure 1 (IMAGE)
Caption
MA-5 treatment comprehensively improves Barth syndrome pathology. In Barth syndrome models (left panel, A.), iPS cell-derived myoblasts show decreased mitochondrial function and increased endoplasmic reticulum (ER) stress, while Drosophila exhibit reduced motor function and elevated heart rate. MA-5 treatment (right panel, B.) restores mitochondrial function, reduces ER stress in myoblasts, and improves both motor function and heart rate in Drosophila models.
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©Yoshiyasu Tongu et al.
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