Skip to main content
Biochemical Journal logoLink to Biochemical Journal
. 1972 Feb;126(4):1035–1037. doi: 10.1042/bj1261035

Tiglicaciduria in propionicacidaemia

William L Nyhan 1, Toshiyuki Ando 1, Karsten Rasmussen 1, William Wadlington 1, Anthony W Kilroy 1, Dennis Cottom 1, David Hull 1
PMCID: PMC1178511  PMID: 5073234

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.

Full text

PDF
1036

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ando T., Rasmussen K., Nyhan W. L., Donnell G. N., Barnes N. D. Propionic acidemia in patients with ketotic hyperglycinemia. J Pediatr. 1971 May;78(5):827–832. doi: 10.1016/s0022-3476(71)80354-2. [DOI] [PubMed] [Google Scholar]
  2. CHILDS B., NYHAN W. L., BORDEN M., BARD L., COOKE R. E. Idiopathic hyperglycinemia and hyperglycinuria: a new disorder of amino acid metabolism. I. Pediatrics. 1961 Apr;27:522–538. [PubMed] [Google Scholar]
  3. Gompertz D., Storrs C. N., Bau D. C., Peters T. J., Hughes E. A. Localisation of enzymic defect in propionicacidaemia. Lancet. 1970 May 30;1(7657):1140–1143. doi: 10.1016/s0140-6736(70)91216-x. [DOI] [PubMed] [Google Scholar]
  4. Hommes F. A., Kuipers J. R., Elema J. D., Jansen J. F., Jonxis J. H. Propionicacidemia, a new inborn error of metabolism. Pediatr Res. 1968 Nov;2(6):519–524. doi: 10.1203/00006450-196811000-00010. [DOI] [PubMed] [Google Scholar]
  5. Menkes J. H. Idiopathic hyperglycinemia: isolation and identification of three previously undescribed urinary ketones. J Pediatr. 1966 Sep;69(3):413–421. doi: 10.1016/s0022-3476(66)80086-0. [DOI] [PubMed] [Google Scholar]
  6. RENDINA G., COON M. J. Enzymatic hydrolysis of the coenzyme a thiol esters of beta-hydroxypropionic and beta-hydroxyisobutyric acids. J Biol Chem. 1957 Mar;225(1):523–534. [PubMed] [Google Scholar]
  7. STERN J. R., DEL CAMPILLO A. Enzymes of fatty acid metabolism. II. Properties of crystalline crotonase. J Biol Chem. 1956 Feb;218(2):985–1002. [PubMed] [Google Scholar]

Articles from Biochemical Journal are provided here courtesy of The Biochemical Society

RESOURCES