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N-Acetylaspartate Synthase Deficiency Corrects the Myelin Phenotype in a Canavan Disease Mouse Model But Does Not Affect Survival Time

Canavan disease (CD) is a severe, lethal leukodystrophy caused by deficiency in aspartoacylase (ASPA), which hydrolyzes N-acetylaspartate (NAA). In the brains of CD patients, NAA accumulates to high millimolar concentrations. The pathology of the disease is characterized by loss of oligodendrocytes...

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Bibliografiska uppgifter
I publikationen:J Neurosci
Huvudupphovsmän: Maier, Helena, Wang-Eckhardt, Lihua, Hartmann, Dieter, Gieselmann, Volkmar, Eckhardt, Matthias
Materialtyp: Artigo
Språk:Inglês
Publicerad: Society for Neuroscience 2015
Ämnen:
Länkar:https://ncbi.nlm.nih.gov/pmc/articles/PMC6605458/
https://ncbi.nlm.nih.gov/pubmed/26511242
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.1056-15.2015
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