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The advantages of this sensitive technique are that the bimane derivatives have a high fluorescence yield and are quite stable (up to several days in solutions kept in the dark), and that other thiols present in the extract, such as cysteine, can be separated and measured in the same run with glutathione (Figure 2A, top)

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Based on the age of onset, LS is classified into early-onset (before 2 years of age) and late-onset (after 2 years)

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Clinical use typically ranges between 5001,000 mg per day

Molecular mechanisms of dominant expression in porphyria