Excretion of S-Adenosylmethionine and S-Adenosylhomocysteine in Homocystinuria

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Abstract
To the Editor: Patients with homocystinuria have increased blood and urinary concentrations of homocystine and methionine.1 The relation, if any, between the elevated homocystine or methionine levels and the clinical symptoms is still not known. Possibilities include the presence of high intracellular concentrations of intermediates or urinary losses of some essential metabolites. During normal metabolism of L-methionine, two adenosylated compounds, S-adenosylmethionine (SAM) and S-adenosylhomocysteine (SAH), are formed intracellularly, and these compounds exist in substantially increased concentrations in experimentally induced hypermethioninemia.2 The possibility that substantial amounts of adenosine, conjugated, in the form of SAM or SAH, would be excreted in the . . .