Homolanthionine Excretion in Homocystinuria

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Science  24 Jun 1966:
Vol. 152, Issue 3730, pp. 1750-1752
DOI: 10.1126/science.152.3730.1750


Patients with homocystinuria excrete in their urine small amounts of an amino acid indistinguishable from authentic L-homolanthionine. This compound could be formed from homocysteine and homoserine by a condensation analogous to that normally leading to cystathionine. The only other known occurrence of homolanthionine in nature is in a methionine-requiring mutant strain of Escherichia coli.