N-acetylaspartylglutamate in Canavan disease: an adverse effector?

@article{Burlina1999NacetylaspartylglutamateIC,
  title={N-acetylaspartylglutamate in Canavan disease: an adverse effector?},
  author={A Burlina and Vera Ferrari and Priscille Divry and Wanda Gradowska and Cornelis Jakobs and Mark J. Bennett and Adrian Clive Sewell and Carlo Dionisi-Vici and Alberto B. Burlina},
  journal={European Journal of Pediatrics},
  year={1999},
  volume={158},
  pages={406-409}
}
We measured N-acetylaspartate and its precursor/product N-acetylaspartylglutamate (NAAG) in urine of patients with Canavan disease using capillary zone electrophoresis. Abnormal levels of NAAG were found in 32 of 43 patients examined. Elevated NAAG was also present in the CSF of one patient. Given that NAAG may interfere with N-methyl-D-aspartate receptor function, the occurrence of high levels of NAAG in patients' urine conceivably represents a participating factor in the pathogenesis of… CONTINUE READING
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