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Seizures and EEG abnormalities are commonly observed in patients with PA and may be related to the neurologic effects of hyperammonemia and the heightened sensitivity of the basal ganglia to metabolic disturbances [6, 19]
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Approximately 2% of the oxygen consumed by the mitochondria is converted into superoxide, making it one of the largest sources of endogenous ROS ( via -oxidation of fatty acid and flavin oxidase reaction and degrading ROS via catalase-mediated breakdown of H 2 O 2 ( Table 1 Formation of major oxidants
The plasma concentration of ATP was quantified using the ATPlite luminescence assay system (PerkinElmer, MA) as we have reported elsewhere (85)
30 mg divided by 4 mg = 7.5 weeks
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