N-acetylaspartylglutamate in Canavan disease: An adverse effector?

A. P. Burlina, V. Ferrari, P. Divry, W. Gradowska, C. Jakobs, M. J. Bennett, A. C. Sewell, C. Dionisi-Vici, A. B. Burlina

Research output: Contribution to journalArticlepeer-review


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 Canavan disease. Conclusion The biochemical role of N-acetylaspartylglutamate and its relationship to glutamatergic function may be relevant to the pathogenesis of Canavan disease.

Original languageEnglish
Pages (from-to)406-409
Number of pages4
JournalEuropean Journal of Pediatrics
Issue number5
Publication statusPublished - 1999


  • Canavan disease
  • Capillary zone electrophoresis
  • N-acetylaspartate
  • N-acetylaspartylglutamate
  • Urine

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health


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