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These key enzymes convert glutamate to GABA

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BH4 acts both as a direct trap for small molecule free radicals and a cofactor for enzymes involved in CoQ10 synthesis, protecting phospholipids containing two polyunsaturated fatty acid (PUFA) tails from oxidative degradation during ferroptosis

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It converts uridine into pseudouridine in both mitochondria and cytosol, thus the clinical findings of both a mitochondrial myopathy and an autosomal recessive disorder

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