Absolute configuration of N-acetylaspartate in urine from patients with Canavan disease.
High-resolution 1H and 13C NMR of N-acetylaspartic acid as its 2-(S)-butyl diester allows the two enantiomers to be unambiguously identified. Analysis of urine samples from five patients with Canavan disease (N-aspartoacylase deficiency) showed that more than 95% of the excreted N-acetylaspartate had the S-configuration.
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Warsaw University of Technology, Faculty of Chemistry, Warsaw, Poland. domibal@yahoo.co.uk
Gryff-Keller
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Gradowska
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D001224:Aspartic Acid / Q000031:analogs & derivatives* / Q000737:chemistry / Q000652:urine; D017825:Canavan Disease / Q000652:urine*; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008968:Molecular Conformation; D013237:Stereoisomerism
D001224:Aspartic Acid; C000179:N-acetylaspartate