N-acetylaspartylglutamate in Canavan disease : an adverse effector?

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 t...

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Bibliographic Details
Published in:European journal of pediatrics 1999-05, Vol.158 (5), p.406-409
Main Authors: BURLINA, A. P, FERRARI, V, DIVRY, P, GRADOWSKA, W, JAKOBS, C, BENNETT, M. J, SEWELL, A. C, DIONISI-VICI, C, BURLINA, A. B
Format: Article
Language:English
Subjects:
Adolescent
Biological and medical sciences
Canavan Disease - cerebrospinal fluid
Canavan Disease - urine
Capillary electrophoresis
Child
Child, Preschool
Chromatography
Creatinine
Dipeptides - cerebrospinal fluid
Dipeptides - urine
Disease
Electrophoresis, Capillary
Enzymes
Errors of metabolism
Humans
Infant
Infant, Newborn
Lipids (lysosomal enzyme disorders, storage diseases)
Mass spectrometry
Medical sciences
Metabolic diseases
Metabolism
Neuropeptides - cerebrospinal fluid
Neuropeptides - urine
Pathogenesis
Patients
Pediatrics
Scientific imaging
Urine
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Summary: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. The biochemical role of N-acetylaspartylglutamate and its relationship to glutamatergic function may be relevant to the pathogenesis of Canavan disease.
ISSN:0340-6199
1432-1076
DOI:10.1007/s004310051102