Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model

Fuzheng Guo, Peter Bannerman, Emily Mills Ko, Laird Miers, Jie Xu, Travis Burns, Shuo Li, Ernest Freeman, Jennifer A. McDonough, David Pleasure

Research output: Contribution to journalArticle

24 Scopus citations

Abstract

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. Ann Neurol 2015;77:884-888

Original languageEnglish (US)
Pages (from-to)884-888
Number of pages5
JournalAnnals of Neurology
Volume77
Issue number5
DOIs
StatePublished - May 1 2015

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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