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

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  3. Ablating the Transporter Sodium-Dependent Dicarboxylate Transporter 3 Prevents Leukodystrophy in Canavan Disease Mice
Author(s)
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
Format
Article
Identifier(s)
10.1002/ana.26211
Publication Date
2021-09-08
Publication Title
Annals of Neurology
Publisher
Wiley
Volume
90
Issue
5
First Page
845
Last Page
850
Rights
http://rightsstatements.org/vocab/InC/1.0/
Community
Healthy Communities Research Institute
Permalink https://oaks.kent.edu/hcri/ablating-transporter-sodium-dependent-dicarboxylate-transporter-3-prevents-leukodystrophy
Wang, Y., Hull, V., Sternbach, S., Popovich, B., Burns, T., McDonough, J., Guo, F., & Pleasure, D. (2021). Ablating the Transporter Sodium-Dependent Dicarboxylate Transporter 3 Prevents Leukodystrophy in Canavan Disease Mice (1–). Annals of Neurology. https://doi.org/http://dx.doi.org/10.1002/ana.26211
Wang, Yan, Vanessa Hull, Sarah Sternbach, Brad Popovich, Travis Burns, Jennifer McDonough, Fuzheng Guo, and David Pleasure. 2021. “Ablating the Transporter Sodium-Dependent Dicarboxylate Transporter 3 Prevents Leukodystrophy in Canavan Disease Mice”. Annals of Neurology. https://doi.org/http://dx.doi.org/10.1002/ana.26211.
Wang, Yan, et al. Ablating the Transporter Sodium-Dependent Dicarboxylate Transporter 3 Prevents Leukodystrophy in Canavan Disease Mice. Annals of Neurology, 8 Sept. 2021, https://doi.org/http://dx.doi.org/10.1002/ana.26211.