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 E Pleasure

Research output: Contribution to journalArticle

21 Citations (Scopus)

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

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Canavan Disease
Central Nervous System
Acetyltransferases
Mutation
N-acetylaspartate

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model. / Guo, Fuzheng; Bannerman, Peter; Mills Ko, Emily; Miers, Laird; Xu, Jie; Burns, Travis; Li, Shuo; Freeman, Ernest; McDonough, Jennifer A.; Pleasure, David E.

In: Annals of Neurology, Vol. 77, No. 5, 01.05.2015, p. 884-888.

Research output: Contribution to journalArticle

Guo, F, Bannerman, P, Mills Ko, E, Miers, L, Xu, J, Burns, T, Li, S, Freeman, E, McDonough, JA & Pleasure, DE 2015, 'Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model', Annals of Neurology, vol. 77, no. 5, pp. 884-888. https://doi.org/10.1002/ana.24392
Guo, Fuzheng ; Bannerman, Peter ; Mills Ko, Emily ; Miers, Laird ; Xu, Jie ; Burns, Travis ; Li, Shuo ; Freeman, Ernest ; McDonough, Jennifer A. ; Pleasure, David E. / Ablating N-acetylaspartate prevents leukodystrophy in a Canavan disease model. In: Annals of Neurology. 2015 ; Vol. 77, No. 5. pp. 884-888.
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