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Tiglicaciduria in propionicacidaemia |
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| Authors: | William L. Nyhan Toshiyuki Ando Karsten Rasmussen William Wadlington Anthony W. Kilroy Dennis Cottom David Hull |
| Affiliation: | Department of Pediatrics, University of California, San Diego, P.O. Box 109, La Jolla, Calif. 92037, U.S.A., Department of Pediatrics, Vanderbilt University, Nashville, Tenn. 37203, U.S.A., and The Hospital for Sick Children, Great Ormond Street, London W.C.1, U.K. |
| Abstract: | Tiglic acid, which has not previously been found in human body fluids, was recently detected in the urine of two patients with propionicacidaemia. These patients had a documented defect in the oxidation of propionate. A competition between acrylyl-CoA and tiglyl-CoA for crotonase could explain the accumulation of tiglic acid. |
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