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l-cystine ?921 amino acid cysteine redox endoplasmic reticulum golgi apparatus lysosomes vesicles cystine-based disorder cystinuria cystinosis cystine hair nutritional supplement skin elasticity n-acetyl-cysteine dicysteine molecule peptide chain therapy 3d metabolite metabolism biosynthesis cycle mechanism pathway path catabolism biosynthetic intermediate biogenesis substrate precursor synthesis derivative bioprecursor metabolic enzyme endogenous contractor human metabolome database molecular structural structure formula skeletal geometry ball stick