Redirecting N-acetylaspartate metabolism in the central nervous system normalizes myelination and rescues Canavan disease
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چکیده
منابع مشابه
Increasing N-acetylaspartate in the Brain during Postnatal Myelination Does Not Cause the CNS Pathologies of Canavan Disease
Canavan disease is caused by mutations in the gene encoding aspartoacylase (ASPA), a deacetylase that catabolizes N-acetylaspartate (NAA). The precise involvement of elevated NAA in the pathogenesis of Canavan disease is an ongoing debate. In the present study, we tested the effects of elevated NAA in the brain during postnatal development. Mice were administered high doses of the hydrophobic m...
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ژورنال
عنوان ژورنال: JCI Insight
سال: 2017
ISSN: 2379-3708
DOI: 10.1172/jci.insight.90807