A convenient experimental animal model of cystathioninuria was obtained by the injection of propargylglycine into rats. The concentrations of propargylglycine at different times after the injection were measured in various tissues, urine, and serum by isotachophoresis. Cystathionine and its derivatives were also measured in several parenchymal tissues. The propargylglycine accumulated rapidly in various tissues and serum of the rats, and the content reached its maximum at about 2h after injection. The urinary excretion of propargylglycine was about 18% of the administered dose. Cystathionine metabolites, such as S-(3-hydroxy-3-carboxy-
n-propyl)cysteine, S-(2-carboxyethyl)cysteine, S-(2-hydroxy-2-carboxyethyl)homocysteine, S-(carboxymethyl)homocysteine, N-acetylcystathionine, N-acetyl-S-(2-carboxyethyl)cysteine, and N-acetyl-S-(3-hydroxy-3-carboxy-
n-propyl)cysteine, were also identified in various tissues of propargylglycine-treated rats.
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