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Cystine appeared to be the main regulator of GSH homeostasis (among other amino acids), since cystine deprivation alone reduced intracellular GSH and it was not further aggravated by depletion of other amino acids
Activating pathways for creation of NADPH has been shown to protect RPE cells from oxidative damage 74 , and our findings indicate that for developing mouse retinas with RP in which all rods have been lost a possible intervention strategy would be to stimulate synthesis of NADPH
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Metabolic consequences of CACT deficiency are hypoketotic hypoglycemia, dicarboxylic aciduria, elevated serum levels of long-chain acylcarnitines, reduced serum carnitine, and hyperammonemia
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