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

Sankar Surendran; Edward L. Ezell; Michael J. Quast; Jingna Wei; Stephen K. Tyring; Kimberlee Michals-Matalon; Reuben Matalon

doi:10.1016/j.brainres.2004.05.035

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

Sankar Surendran, Edward L. Ezell, Michael J. Quast, Jingna Wei, Stephen K. Tyring, Kimberlee Michals-Matalon, Reuben Matalon

Pediatrics

Research output: Contribution to journal › Article › peer-review

12 Scopus citations

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.

Original language	English (US)
Pages (from-to)	268-271
Number of pages	4
Journal	Brain Research
Volume	1016
Issue number	2
DOIs	https://doi.org/10.1016/j.brainres.2004.05.035
State	Published - Aug 6 2004

Keywords

ASPA gene knockout mouse
Canavan disease
Degenerative disease: other
Disorders of the nervous system
Glutamate carboxypeptidase II
N-Acetylaspartylglutamate

ASJC Scopus subject areas

General Neuroscience
Molecular Biology
Clinical Neurology
Developmental Biology

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10.1016/j.brainres.2004.05.035

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

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.",

keywords = "ASPA gene knockout mouse, Canavan disease, Degenerative disease: other, Disorders of the nervous system, Glutamate carboxypeptidase II, N-Acetylaspartylglutamate",

author = "Sankar Surendran and Ezell, {Edward L.} and Quast, {Michael J.} and Jingna Wei and Tyring, {Stephen K.} and Kimberlee Michals-Matalon and Reuben Matalon",

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AU - Surendran, Sankar

AU - Ezell, Edward L.

AU - Quast, Michael J.

AU - Wei, Jingna

AU - Tyring, Stephen K.

AU - Michals-Matalon, Kimberlee

AU - Matalon, Reuben

PY - 2004/8/6

Y1 - 2004/8/6

N2 - 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.

AB - 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.

KW - ASPA gene knockout mouse

KW - Canavan disease

KW - Degenerative disease: other

KW - Disorders of the nervous system

KW - Glutamate carboxypeptidase II

KW - N-Acetylaspartylglutamate

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

Abstract

Keywords

ASJC Scopus subject areas

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