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Therefore, ornithine and proline are both glucogenic

At this stage in the deficiency all four chambers of the heart enlarge due to loss of energy generation and fluid retention resulting in what is called dilated cardiomyopathy

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In addition to platelet modulation, decreased levels of GSH increases local ROS in endothelial tissue contributing to a decrease in NO, an upregulation of NF- B, and subsequent increase in cytokine production and prothrombotic adhesion molecules