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KD has been shown to enhance intestinal stem cell self-renewal 20 and increase the proliferation of muscle satellite cells in Duchenne disease 21

Oh, M.-H

Long-term and sustained mitochondrial dysfunction Impairment of energy metabolism, secondary to chronic mitochondrial dysfunction due to mechanisms such as toxic metabolites direct damage, deficiency of essential metabolic intermediates, and to concomitantly increased respiratory oxidative stress, is thought to play a crucial role in the development of both cardiomyopathy and LQTS in patients with PA (Figure 2)

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