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Science 24 June 1966:
Vol. 152. no. 3730, pp. 1750 - 1752
DOI: 10.1126/science.152.3730.1750
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Articles

Homolanthionine Excretion in Homocystinuria

Thomas L. Perry 1, Shirley Hansen 1, and Lynne MacDougall 1

1 Department of Pharmacology, University of British Columbia, Vancouver 8, Canada

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.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
H2S Biogenesis by Human Cystathionine {gamma}-Lyase Leads to the Novel Sulfur Metabolites Lanthionine and Homolanthionine and Is Responsive to the Grade of Hyperhomocysteinemia.
T. Chiku, D. Padovani, W. Zhu, S. Singh, V. Vitvitsky, and R. Banerjee (2009)
J. Biol. Chem. 284, 11601-11612
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Science. ISSN 0036-8075 (print), 1095-9203 (online)