These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Urinary excretion of homocitric acid and methylhomocitric acid in propionic acidaemia: minor metabolic products of the citrate synthase aldol condensation reaction.
    Author: van Rooyen JP, Mienie LJ, Erasmus E, de Wet WJ, Duran M, Wadman SK.
    Journal: Clin Chim Acta; 1994 Oct 14; 230(1):91-9. PubMed ID: 7850997.
    Abstract:
    The identity of two formerly novel citric acid analogues, homocitric acid and methylhomocitric acid, in urine samples from patients with propionic acidaemia was confirmed by gas chromatography and mass spectrometry. Authentic reference substances were synthesized using a Reformatskii reaction. Homocitric acid and methylhomocitric acid were detected as minor metabolites in the urine samples from propionyl coenzyme A carboxylase deficient individuals. It was shown that these substances can be formed by the citrate synthase condensation reaction of alpha-ketoglutarate with acetyl coenzyme A and propionyl coenzyme A, respectively.
    [Abstract] [Full Text] [Related] [New Search]