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

doi:10.1002/ana.26211

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

Neurology

Research output: Contribution to journal › Article › peer-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 language	English (US)
Journal	Annals of Neurology
DOIs	https://doi.org/10.1002/ana.26211
State	Accepted/In press - 2021

ASJC Scopus subject areas

Neurology
Clinical Neurology

Access to Document

10.1002/ana.26211

Cite this

@article{2ce010b41d15421e88967f59bac3a4ba,

title = "Ablating the Transporter Sodium-Dependent Dicarboxylate Transporter 3 Prevents Leukodystrophy in Canavan Disease Mice",

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.",

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

note = "Funding Information: This study was supported by NIH 1R21117386 (DP) and UL1 TR001860 (VH); Shriners grants 84553‐NCA‐18 (YW), 87400‐NCA‐21 (YW), and 85113‐NCA‐18 (DP); and the Dana Foundation (DP). Publisher Copyright: {\textcopyright} 2021 American Neurological Association.",

year = "2021",

doi = "10.1002/ana.26211",

language = "English (US)",

journal = "Annals of Neurology",

issn = "0364-5134",

publisher = "John Wiley and Sons Inc.",

}

TY - JOUR

T1 - Ablating the Transporter Sodium-Dependent Dicarboxylate Transporter 3 Prevents Leukodystrophy in Canavan Disease Mice

AU - Wang, Yan

AU - Hull, Vanessa

AU - Sternbach, Sarah

AU - Popovich, Brad

AU - Burns, Travis

AU - McDonough, Jennifer

AU - Guo, Fuzheng

AU - Pleasure, David

N1 - Funding Information: This study was supported by NIH 1R21117386 (DP) and UL1 TR001860 (VH); Shriners grants 84553‐NCA‐18 (YW), 87400‐NCA‐21 (YW), and 85113‐NCA‐18 (DP); and the Dana Foundation (DP). Publisher Copyright: © 2021 American Neurological Association.

PY - 2021

Y1 - 2021

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=85115384626&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85115384626&partnerID=8YFLogxK

U2 - 10.1002/ana.26211

DO - 10.1002/ana.26211

M3 - Article

AN - SCOPUS:85115384626

JO - Annals of Neurology

JF - Annals of Neurology

SN - 0364-5134

ER -

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

Abstract

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this