N-acetylaspartylglutamate in Canavan disease: an adverse effector?

Abstract

UNLABELLED:

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.

• Authors: A P Burlina, V Ferrari, P Divry, W Gradowska, C Jakobs, M J Bennett, A C Sewell, C Dionisi-Vici, A B Burlina
• Journal: European journal of pediatrics (Eur J Pediatr) Vol. 158 Issue 5 Pg. 406-9 (May 1999) ISSN: 0340-6199 [Print] Germany
• PMID: 10333125 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Dipeptides
• Neuropeptides
• isospaglumic acid

Topics

• Adolescent
• Canavan Disease (cerebrospinal fluid, urine)
• Child
• Child, Preschool
• Dipeptides (cerebrospinal fluid, urine)
• Electrophoresis, Capillary
• Humans
• Infant
• Infant, Newborn
• Neuropeptides (cerebrospinal fluid, urine)