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It is postulated that glutathione inhibits tyrosinase through three primary mechanisms: direct antioxidant activity, chelation of the copper site within tyrosinase via its thiol group, and disruption of tyrosinase's cellular transport to pre-melanosomes

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Prognosis is very poor with a life expectancy ranging from 3 to 5 years from presentation of first symptoms, with the majority of deaths resulting from respiratory insufficiency (Niedermeyer et al., 2019

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