Aspartoacylase deficiency does not affect N-acetylaspartylglutamate level or glutamate carboxypeptidase II activity in the knockout mouse brain

Aspartoacylase (ASPA)-deficient patients [Canavan disease (CD)] reportedly have increased urinary excretion of N-acetylaspartylglutamate (NAAG), a neuropeptide abundant in the brain. Whether elevated excretion of urinary NAAG is due to ASPA deficiency, resulting in an abnormal level of brain NAAG, i...

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Bibliographic Details
Published in:Brain research 2004-08, Vol.1016 (2), p.268-271
Main Authors: Surendran, Sankar, Ezell, Edward L, Quast, Michael J, Wei, Jingna, Tyring, Stephen K, Michals-Matalon, Kimberlee, Matalon, Reuben
Format: Article
Language:English
Subjects:
Amidohydrolases - deficiency
Amidohydrolases - genetics
Animals
ASPA gene knockout mouse
Biological and medical sciences
Brain - anatomy & histology
Brain - enzymology
Brain - metabolism
Brain Chemistry - genetics
Canavan disease
Dipeptides - metabolism
Errors of metabolism
Glutamate carboxypeptidase II
Glutamate Carboxypeptidase II - metabolism
Lipids (lysosomal enzyme disorders, storage diseases)
Magnetic Resonance Spectroscopy - methods
Medical sciences
Metabolic diseases
Mice
Mice, Knockout - physiology
N-Acetylaspartylglutamate
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Summary:Aspartoacylase (ASPA)-deficient patients [Canavan disease (CD)] reportedly have increased urinary excretion of N-acetylaspartylglutamate (NAAG), a neuropeptide abundant in the brain. Whether elevated excretion of urinary NAAG is due to ASPA deficiency, resulting in an abnormal level of brain NAAG, is examined using ASPA-deficient mouse brain. The level of NAAG in the knockout mouse brain was similar to that in the wild type. The NAAG hydrolyzing enzyme, glutamate carboxypeptidase II (GCP II), activity was normal in the knockout mouse brain. These data suggest that ASPA deficiency does not affect the NAAG or GCP II level in the knockout mouse brain, if documented also in patients with CD.
ISSN:0006-8993
1872-6240
DOI:10.1016/j.brainres.2004.05.035