Aminoaciduria

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Abstract
Disorders of Methionine MetabolismThe normal pathway of methionine metabolism is shown in Figure 3. Methionine has at least two major roles in addition to that in protein synthesis: it is the major methyl donor of the body and supplies methyl groups for synthesis of many compounds; and it is a precursor of cysteine and cystine. When there is a block in the metabolic pathway from methionine to cysteine, the plasma and urine cystine and taurine may be abnormally low.At least two inborn errors of methionine metabolism have been described thus far. They are named after the accumulated amino acid . . .

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