EXCRETION OF 3,6-EPOXYDICARBOXYLIC ACIDS IN PEROXISOMAL DISORDERS

Authors
Citation
Jj. Pitt et A. Poulos, EXCRETION OF 3,6-EPOXYDICARBOXYLIC ACIDS IN PEROXISOMAL DISORDERS, Clinica chimica acta, 223(1-2), 1993, pp. 23-29
Citations number
16
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Chemistry Medicinal
Journal title
ISSN journal
0009-8981
Volume
223
Issue
1-2
Year of publication
1993
Pages
23 - 29
Database
ISI
SICI code
0009-8981(1993)223:1-2<23:EO3AIP>2.0.ZU;2-H
Abstract
The urinary excretions of several organic acids were quantitatively st udied by gas chromatography/mass spectrometry in subjects with disorde rs of peroxisome biogenesis (n = 8) and controls (n = 26). The excreti on of 3,6-epoxytetradecanedioic acid was significantly elevated in all subjects with disorders of peroxisome biogenesis (1.8-20.8; controls, not detected-0.5, mu mol/mmol of creatinine). 3,6-Epoxydodecanedioic acid excretion was usually elevated (1.4-19.8; controls, not detected- 4.2) and 3,6-epoxyoctanedioic acid excretion was not elevated not dete cted-8.8; controls, 0.6-9.5 nu mol/mmol of creatinine). It is suggeste d that measurement of 3,6-epoxydicarboxylic acids may be useful for th e diagnosis of these disorders.