J Inherit Metab Dis. 2005;28(4):607-9.

Absolute configuration of N-acetylaspartate in urine from patients with Canavan disease.

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Warsaw University of Technology, Faculty of Chemistry, Warsaw, Poland. domibal@yahoo.co.uk

Abstract

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.

PMID:: 15902566; [PubMed - indexed for MEDLINE]

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Aspartoacylase deficiency and N-acetylaspartic aciduria in patients with Canavan disease. [Am J Med Genet. 1988]
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