Homolanthionine excretion in homocystinuria.
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.
D000293:Adolescent; D000592:Amino Acid Metabolism, Inborn Errors; D000596:Amino Acids; D055598:Chemical Phenomena; D002621:Chemistry; D002627:Chemistry, Physical; D002648:Child; D002675:Child, Preschool; D002845:Chromatography; D002854:Chromatography, Paper; D006710:Homocysteine / Q000378:metabolism*; D006801:Humans; D066298:In Vitro Techniques; D014556:Urine
D000596:Amino Acids; D006710:Homocysteine