Transport of N-acetylaspartate via murine sodium/dicarboxylate cotransporter NaDC3 and expression of this transporter and aspartoacylase II in ocular tissues in mouse

Ronald L. George, Wei Huang, Hany A. Naggar, Sylvia B Smith, Vadivel Ganapathy

Research output: Contribution to journalArticle

25 Citations (Scopus)

Abstract

Canavan disease is a genetic disorder associated with optic neuropathy and the metabolism of N-acetylaspartate is defective in this disorder due to mutations in the gene coding for the enzyme aspartoacylase II. Here we show that the plasma membrane transporter NaDC3, a Na+-coupled transporter for dicarboxylates, is able to transport N-acetylaspartate, suggesting that the transporter may function in concert with aspartoacylase II in the metabolism of N-acetylaspartate. Since Canavan disease is associated with ocular complications, we investigated the expression pattern of NaDC3 and aspartoacylase II in ocular tissues in mouse by in situ hybridization. These studies show that NaDC3 mRNA is expressed in the optic nerve, most layers of the retina, retinal pigment epithelium, ciliary body, iris, and lens. Aspartoacylase II mRNA is coexpressed in most of these cell types. We conclude that transport of N-acetylaspartate into ocular tissues via NaDC3 and its subsequent hydrolysis by aspartoacylase II play an essential role in the maintenance of visual function.

Original languageEnglish (US)
Pages (from-to)63-69
Number of pages7
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Volume1690
Issue number1
DOIs
StatePublished - Sep 6 2004

Fingerprint

Sodium
Canavan Disease
Dicarboxylic Acid Transporters
Optic Nerve Diseases
Messenger RNA
Ciliary Body
Inborn Genetic Diseases
Membrane Transport Proteins
Retinal Pigment Epithelium
Iris
Optic Nerve
Lenses
In Situ Hybridization
Retina
Hydrolysis
Maintenance
Cell Membrane
aspartoacylase
N-acetylaspartate
Mutation

Keywords

  • Aspartoacyalse II
  • Canavan disease
  • N-Acetylaspartate
  • NaDC3
  • Optic neuropathy
  • RPE
  • Retina
  • Sodium/dicarboxylate transporter

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology

Cite this

Transport of N-acetylaspartate via murine sodium/dicarboxylate cotransporter NaDC3 and expression of this transporter and aspartoacylase II in ocular tissues in mouse. / George, Ronald L.; Huang, Wei; Naggar, Hany A.; Smith, Sylvia B; Ganapathy, Vadivel.

In: Biochimica et Biophysica Acta - Molecular Basis of Disease, Vol. 1690, No. 1, 06.09.2004, p. 63-69.

Research output: Contribution to journalArticle

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