Simultaneous enantioselective analysis of chiral urinary metabolites in patients with Zellweger syndrome.
Enantio-MDGC-MS analysis with heptakis-(2,3-di-O-methyl-6-O-tert.-butyl-dimethylsilyl)-beta-cyclodextrin as the chiral main column is a powerful tool for the separation of chiral compounds. This paper reports on the simultaneous stereodifferentiation of 2-hydroxyisocaproic acid (HICA), 3-methyladipic acid (3-MA), 2-hydroxyglutaric acid (2-HG), 3-(4-hydroxyphenyl)-lactic acid (HPLA), 2-hydroxysebacic acid (2-HS) and 3-hydroxysebacic acid (3-HS) in a single chromatographic run. These chiral urinary metabolites are useful in the diagnosis of peroxisomal diseases such as Zellweger syndrome (ZS). In this investigation, urine samples from nine patients with ZS were analysed in order to reveal the enantiomeric ratio of these chiral metabolites. The stereodifferentiation of the analysed chiral compounds may provide important information on their biochemical origin.
Muth
Alexandra
A
Institut für Lebensmittelchemie, Biozentrum J. W. Goethe-Universität, Marie-Curie-Strasse 9, D-60439 Frankfurt am Main, Germany.
Jung
Jochen
J
Bilke
Steffi
S
Scharrer
Annette
A
Mosandl
Armin
A
Sewell
Adrian C
AC
Böhles
Hans
H
D002264:Carboxylic Acids / Q000652:urine; D002851:Chromatography, High Pressure Liquid; D003652:Decanoic Acids / Q000652:urine; D003998:Dicarboxylic Acids; D008401:Gas Chromatography-Mass Spectrometry; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D013237:Stereoisomerism; D015211:Zellweger Syndrome / Q000652:urine*
D002264:Carboxylic Acids; D003652:Decanoic Acids; D003998:Dicarboxylic Acids; C011107:sebacic acid