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Publication : Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model.

First Author  Guo F Year  2015
Journal  Ann Neurol Volume  77
Issue  5 Pages  884-8
PubMed ID  25712859 Mgi Jnum  J:339025
Mgi Id  MGI:7519247 Doi  10.1002/ana.24392
Citation  Guo F, et al. (2015) Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model. Ann Neurol 77(5):884-8
abstractText  Canavan disease is caused by inactivating ASPA (aspartoacylase) mutations that prevent cleavage of N-acetyl-L-aspartate (NAA), resulting in marked elevations in central nervous system (CNS) NAA and progressively worsening leukodystrophy. We now report that ablating NAA synthesis by constitutive genetic disruption of Nat8l (N-acetyltransferase-8 like) permits normal CNS myelination and prevents leukodystrophy in a murine Canavan disease model.
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