URINARY-EXCRETION OF HOMOCITRIC ACID AND METHYLHOMOCITRIC ACID IN PROPIONIC ACIDEMIA - MINOR METABOLIC PRODUCTS OF THE CITRATE SYNTHASE ALDOL CONDENSATION REACTION

Authors
VANROOYEN JPG MIENIE LJ ERASMUS E DEWET WJ DURAN M WADMAN SK
Citation
Jpg. Vanrooyen et al., URINARY-EXCRETION OF HOMOCITRIC ACID AND METHYLHOMOCITRIC ACID IN PROPIONIC ACIDEMIA - MINOR METABOLIC PRODUCTS OF THE CITRATE SYNTHASE ALDOL CONDENSATION REACTION, Clinica chimica acta, 230(1), 1994, pp. 91-99
Citations number
5
Categorie Soggetti
Chemistry Medicinal
Journal title
Clinica chimica acta → ACNP
ISSN journal
00098981
Volume
230
Issue
1
Year of publication
1994
Pages
91 - 99
Database
ISI
SICI code
0009-8981(1994)230:1<91:UOHAAM>2.0.ZU;2-W
Abstract
The identity of two formerly novel citric acid analogues, homocitric a cid and methylhomocitric acid, in urine samples from patients with pro pionic acidaemia was confirmed by gas chromatography and mass spectrom etry. Authentic reference substances were synthesized using a Reformat skii reaction. Homocitric acid and methylhomocitric acid were detected as minor metabolites in the urine samples from propionyl coenzyme A c arboxylase deficient individuals. It was shown that these substances c an be formed by the citrate synthase condensation reaction of alpha-ke toglutarate with acetyl coenzyme A and propionyl coenzyme A, respectiv ely.