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FAA, which accumulates in FAH deficiency, is highly electrophilic and a potent alkylator, causing oxidative damage to the cells in which it is generated by reacting with glutathione and sulfhydryl groups of proteins

Glutamate-cysteine ligase catalyzes the first rate-limiting enzymatic step, which is limited by the availability of the sulfhydryl amino acid (AA) L-cysteine (L-cys) (Valdovinos-Flores and Gonsebatt, 2012)

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Glutathione IV side effects are minimal when administered by trained professionals

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