| First Author | Wang Y | Year | 2021 |
| Journal | Ann Neurol | Volume | 90 |
| Issue | 5 | Pages | 845-850 |
| PubMed ID | 34498299 | Mgi Jnum | J:350376 |
| Mgi Id | MGI:7660896 | Doi | 10.1002/ana.26211 |
| Citation | Wang Y, et al. (2021) Ablating the Transporter Sodium-Dependent Dicarboxylate Transporter 3 Prevents Leukodystrophy in Canavan Disease Mice. Ann Neurol 90(5):845-850 |
| abstractText | 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;90:845-850. |
