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. |