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Absolute configuration of N ‐acetylaspartate in urine from patients with Canavan disease
Author(s) -
Bal D.,
GryffKeller A.,
Gradowska W.
Publication year - 2005
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/s10545-005-0607-7
Subject(s) - urine , absolute configuration , enantiomer , high resolution , chemistry , medicine , stereochemistry , remote sensing , geology
Summary High‐resolution 1 H and 13 C 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.