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

Research output: Contribution to journalArticle

12 Citations (Scopus)

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 languageEnglish (US)
Pages (from-to)268-271
Number of pages4
JournalBrain Research
Volume1016
Issue number2
DOIs
StatePublished - Aug 6 2004

Fingerprint

Glutamate Carboxypeptidase II
Canavan Disease
Knockout Mice
Brain
Neuropeptides
N-acetyl-1-aspartylglutamic acid
Enzymes

Keywords

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

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Aspartoacylase deficiency does not affect N-acetylaspartylglutamate level or glutamate carboxypeptidase II activity in the knockout mouse brain. / Surendran, Sankar; Ezell, Edward L.; Quast, Michael J.; Wei, Jingna; Tyring, Stephen K.; Michals-Matalon, Kimberlee; Matalon, Reuben.

In: Brain Research, Vol. 1016, No. 2, 06.08.2004, p. 268-271.

Research output: Contribution to journalArticle

Surendran, Sankar ; Ezell, Edward L. ; Quast, Michael J. ; Wei, Jingna ; Tyring, Stephen K. ; Michals-Matalon, Kimberlee ; Matalon, Reuben. / Aspartoacylase deficiency does not affect N-acetylaspartylglutamate level or glutamate carboxypeptidase II activity in the knockout mouse brain. In: Brain Research. 2004 ; Vol. 1016, No. 2. pp. 268-271.
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