Uncoupling N-acetylaspartate from brain pathology: implications for Canavan disease gene therapy

N-Acetylaspartate (NAA) is the second most abundant organic metabolite in the brain, but its physiological significance remains enigmatic. Toxic NAA accumulation appears to be the key factor for neurological decline in Canavan disease—a fatal neurometabolic disorder caused by deficiency in the NAA-d...

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Detalhes bibliográficos
Publicado no:Acta Neuropathol
Main Authors: von Jonquieres, Georg, Spencer, Ziggy H. T., Rowlands, Benjamin D., Klugmann, Claudia B., Bongers, Andre, Harasta, Anne E., Parley, Kristina E., Cederholm, Jennie, Teahan, Orla, Pickford, Russell, Delerue, Fabien, Ittner, Lars M., Fröhlich, Dominik, McLean, Catriona A., Don, Anthony S., Schneider, Miriam, Housley, Gary D., Rae, Caroline D., Klugmann, Matthias
Formato: Artigo
Idioma:Inglês
Publicado em: Springer Berlin Heidelberg 2017
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Original Paper
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5756261/
https://ncbi.nlm.nih.gov/pubmed/29116375
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00401-017-1784-9
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