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Abnormal mitochondrial function, specifically mitochondrial dysfunction in the myocardium, fulfills an important role in the progression of cardiomyopathy, and mutations in the GTP-binding protein 3 or mitochondrial translation optimization 1 homolog genes were shown to elicit an impaired assembly of respiratory chain complexes through impairing the 5-taurinomethyluridine (m5U) modification of mitochondrial tRNAs, ultimately leading to HCM (166)

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