Suppressing N-Acetyl-l-Aspartate Synthesis Prevents Loss of Neurons in a Murine Model of Canavan Leukodystrophy

Canavan disease is a leukodystrophy caused by aspartoacylase (ASPA) deficiency. The lack of functional ASPA, an enzyme enriched in oligodendroglia that cleaves N-acetyl-l-aspartate (NAA) to acetate and l-aspartic acid, elevates brain NAA and causes “spongiform” vacuolation of superficial brain white...

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Publicado en:J Neurosci
Main Authors: Sohn, Jiho, Bannerman, Peter, Guo, Fuzheng, Burns, Travis, Miers, Laird, Croteau, Christopher, Singhal, Naveen K., McDonough, Jennifer A., Pleasure, David
Formato: Artigo
Idioma:Inglês
Publicado: Society for Neuroscience 2017
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Research Articles
Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC5242397/
https://ncbi.nlm.nih.gov/pubmed/28077719
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.2013-16.2016
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