Literature DB >> 5073234

Tiglicaciduria in propionicacidaemia.

W L Nyhan, T Ando, K Rasmussen, W Wadlington, A W Kilroy, D Cottom, D Hull.   

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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Year:  1972        PMID： 5073234      PMCID： PMC1178511          DOI： 10.1042/bj1261035

Source DB:  PubMed          Journal:  Biochem J        ISSN： 0264-6021            Impact factor:   3.857


  7 in total

1.  Idiopathic hyperglycinemia and hyperglycinuria: a new disorder of amino acid metabolism. I.

Authors:  B CHILDS; W L NYHAN; M BORDEN; L BARD; R E COOKE
Journal:  Pediatrics       Date:  1961-04       Impact factor: 7.124

2.  Enzymes of fatty acid metabolism. II. Properties of crystalline crotonase.

Authors:  J R STERN; A DEL CAMPILLO
Journal:  J Biol Chem       Date:  1956-02       Impact factor: 5.157

3.  Localisation of enzymic defect in propionicacidaemia.

Authors:  D Gompertz; C N Storrs; D C Bau; T J Peters; E A Hughes
Journal:  Lancet       Date:  1970-05-30       Impact factor: 79.321

4.  Propionicacidemia, a new inborn error of metabolism.

Authors:  F A Hommes; J R Kuipers; J D Elema; J F Jansen; J H Jonxis
Journal:  Pediatr Res       Date:  1968-11       Impact factor: 3.756

5.  Propionic acidemia in patients with ketotic hyperglycinemia.

Authors:  T Ando; K Rasmussen; W L Nyhan; G N Donnell; N D Barnes
Journal:  J Pediatr       Date:  1971-05       Impact factor: 4.406

6.  Idiopathic hyperglycinemia: isolation and identification of three previously undescribed urinary ketones.

Authors:  J H Menkes
Journal:  J Pediatr       Date:  1966-09       Impact factor: 4.406

7.  Enzymatic hydrolysis of the coenzyme a thiol esters of beta-hydroxypropionic and beta-hydroxyisobutyric acids.

Authors:  G RENDINA; M J COON
Journal:  J Biol Chem       Date:  1957-03       Impact factor: 5.157
  7 in total
  3 in total

1.  3-hydroxypropionate: significance of -oxidation of propionate in patients with propionic acidemia and methylmalonic acidemia.

Authors:  T Ando; K Rasmussen; W L Nyhan; D Hull
Journal:  Proc Natl Acad Sci U S A       Date:  1972-10       Impact factor: 11.205

2.  Propionyl-CoA carboxylase deficiency with overflow of metabolites of isoleucine catabolism at all levels.

Authors:  H Przyrembel; H J Bremer; M Duran; L Bruinvis; D Ketting; S K Wadman; R Baumgartner; U Irle; C Bachmann
Journal:  Eur J Pediatr       Date:  1979-01-18       Impact factor: 3.183

3.  Understanding acute metabolic decompensation in propionic and methylmalonic acidemias: a deep metabolic phenotyping approach.

Authors:  H A Haijes; J J M Jans; M van der Ham; P M van Hasselt; N M Verhoeven-Duif
Journal:  Orphanet J Rare Dis       Date:  2020-03-06       Impact factor: 4.123
  3 in total

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