Tiglicaciduria in propionicacidaemia-Reference-Cited by-同舟云学术

Tiglicaciduria in propionicacidaemia

Published:1972-02-01 Issue:4 Volume:126 Page:1035-1037
ISSN:0306-3283
Container-title:Biochemical Journal
language:en
Short-container-title:

Author:

Nyhan William L.¹,Ando Toshiyuki¹,Rasmussen Karsten¹,Wadlington William¹,Kilroy Anthony W.¹,Cottom Dennis¹,Hull David¹

Affiliation:

1. 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.

Publisher

Portland Press Ltd.

Link

https://portlandpress.com/biochemj/article-pdf/126/4/1035/774688/bj1261035.pdf

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