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

Brain Research
Sankar SurendranReuben Matalon

Abstract

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.

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Oct 22, 2003·Molecular Genetics and Metabolism·Sankar SurendranReuben Matalon

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Citations

Nov 4, 2008·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Namik KayaBrian F Meyer
Jun 1, 2011·PloS One·Nadine MersmannMatthias Klugmann
Dec 30, 2006·Proceedings of the National Academy of Sciences of the United States of America·Eduard BittoGeorge N Phillips
May 22, 2009·Journal of Neurochemistry·Alberto Fernández-MedardeEugenio Santos
Jun 30, 2006·Mental Retardation and Developmental Disabilities Research Reviews·Shalini KumarJean de Vellis

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Canavan disease, a type of leukodystrophy, is an autosomal recessive neurodegenerative disorder, and is one of the most common degenerative cerebral diseases of infancy. Discover the latest research on Canavan disease here.