Ablating the Transporter Sodium-Dependent Dicarboxylate Transporter 3 Prevents Leukodystrophy in Canavan Disease Mice

Yan Wang, Vanessa Hull, Sarah Sternbach, Brad Popovich, Travis Burns, Jennifer McDonough, Fuzheng Guo, David Pleasure

Research output: Contribution to journalArticlepeer-review

Abstract

Canavan disease is caused by ASPA mutations that diminish brain aspartoacylase activity, and it is characterized by excessive brain storage of the aspartoacylase substrate, N-acetyl-l-aspartate (NAA), and by astroglial and intramyelinic vacuolation. Astroglia and the arachnoid mater express sodium-dependent dicarboxylate transporter (NaDC3), encoded by SLC13A3, a sodium-coupled transporter for NAA and other dicarboxylates. Constitutive Slc13a3 deletion in aspartoacylase-deficient Canavan disease mice prevents brain NAA overaccumulation, ataxia, and brain vacuolation. ANN NEUROL 2021.

Original languageEnglish (US)
JournalAnnals of Neurology
DOIs
StateAccepted/In press - 2021

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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