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Mammals usually rely solely on extracellular uptake as the primary source of Cys, but some mammals also use cystathionine γ-lyase (CGL)-mediated cystathionine-cleavage in the transsulfuration pathway as a surrogate Cys source if System X c − is blocked (Shimada and Stockwell, 2016)
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Riboflavin supplementation has successfully ameliorated clinical symptoms and metabolic abnormalities in almost all patients (98%) with late-onset MADD, the majority of whom have ETFDH mutations (93%) [138]
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