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Materials and Methods Animals and Experimental Design Male Wistar rats aged 810 weeks and weighing 200250 g were housed in standard laboratory conditions with a temperature of 22 2 C, a 12-hour light/dark cycle, and unrestricted access to food and water

The human body needs 20 muscle-building amino acids, of which nine cannot be produced within the body itself

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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