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

Autor: Wang Y; Institute for Pediatric Regenerative Medicine, UC Davis, c/o Shriners Hospital, Sacramento, CA., Hull V; Institute for Pediatric Regenerative Medicine, UC Davis, c/o Shriners Hospital, Sacramento, CA., Sternbach S; Department of Biological Sciences and School of Biomedical Sciences, Kent State University, Kent, OH., Popovich B; Department of Chemistry and Biochemistry, Kent State University, Kent, OH., Burns T; Institute for Pediatric Regenerative Medicine, UC Davis, c/o Shriners Hospital, Sacramento, CA., McDonough J; Department of Biological Sciences and School of Biomedical Sciences, Kent State University, Kent, OH., Guo F; Institute for Pediatric Regenerative Medicine, UC Davis, c/o Shriners Hospital, Sacramento, CA., Pleasure D; Institute for Pediatric Regenerative Medicine, UC Davis, c/o Shriners Hospital, Sacramento, CA.
Jazyk: angličtina
Zdroj: Annals of neurology [Ann Neurol] 2021 Nov; Vol. 90 (5), pp. 845-850. Date of Electronic Publication: 2021 Sep 24.
DOI: 10.1002/ana.26211
Abstrakt: 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.
(© 2021 American Neurological Association.)
Databáze: MEDLINE
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