Literature DB >> 25712859

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

Fuzheng Guo1, Peter Bannerman, Emily Mills Ko, Laird Miers, Jie Xu, Travis Burns, Shuo Li, Ernest Freeman, Jennifer A McDonough, David Pleasure.   

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.
© 2015 American Neurological Association.

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Year:  2015        PMID： 25712859     DOI： 10.1002/ana.24392

Source DB:  PubMed          Journal:  Ann Neurol        ISSN： 0364-5134            Impact factor:   10.422


  18 in total

1.  Redirecting N-acetylaspartate metabolism in the central nervous system normalizes myelination and rescues Canavan disease.

Authors:  Dominic J Gessler; Danning Li; Hongxia Xu; Qin Su; Julio Sanmiguel; Serafettin Tuncer; Constance Moore; Jean King; Reuben Matalon; Guangping Gao
Journal:  JCI Insight       Date:  2017-02-09

2.  Pathophysiology and Treatment of Canavan Disease.

Authors:  David Pleasure; Fuzheng Guo; Olga Chechneva; Peter Bannerman; Jennifer McDonough; Travis Burns; Yan Wang; Vanessa Hull
Journal:  Neurochem Res       Date:  2018-12-08       Impact factor: 3.996

3.  Suppressing N-Acetyl-l-Aspartate Synthesis Prevents Loss of Neurons in a Murine Model of Canavan Leukodystrophy.

Authors:  Jiho Sohn; Peter Bannerman; Fuzheng Guo; Travis Burns; Laird Miers; Christopher Croteau; Naveen K Singhal; Jennifer A McDonough; David Pleasure
Journal:  J Neurosci       Date:  2017-01-11       Impact factor: 6.167

4.  The neuronal metabolite NAA regulates histone H3 methylation in oligodendrocytes and myelin lipid composition.

Authors:  N K Singhal; H Huang; S Li; R Clements; J Gadd; A Daniels; E E Kooijman; P Bannerman; T Burns; F Guo; D Pleasure; E Freeman; L Shriver; J McDonough
Journal:  Exp Brain Res       Date:  2016-10-05       Impact factor: 1.972

5.  Ablating the Transporter Sodium-Dependent Dicarboxylate Transporter 3 Prevents Leukodystrophy in Canavan Disease Mice.

Authors:  Yan Wang; Vanessa Hull; Sarah Sternbach; Brad Popovich; Travis Burns; Jennifer McDonough; Fuzheng Guo; David Pleasure
Journal:  Ann Neurol       Date:  2021-09-24       Impact factor: 11.274

6.  Brain Nat8l Knockdown Suppresses Spongiform Leukodystrophy in an Aspartoacylase-Deficient Canavan Disease Mouse Model.

Authors:  Peter Bannerman; Fuzheng Guo; Olga Chechneva; Travis Burns; Xiaoqing Zhu; Yan Wang; Bokyung Kim; Naveen K Singhal; Jennifer A McDonough; David Pleasure
Journal:  Mol Ther       Date:  2018-01-10       Impact factor: 11.454

7.  Sox2 Is Essential for Oligodendroglial Proliferation and Differentiation during Postnatal Brain Myelination and CNS Remyelination.

Authors:  Sheng Zhang; Xiaoqing Zhu; Xuehong Gui; Christopher Croteau; Lanying Song; Jie Xu; Aijun Wang; Peter Bannerman; Fuzheng Guo
Journal:  J Neurosci       Date:  2018-01-15       Impact factor: 6.167

8.  Atypical clinical and radiological course of a patient with Canavan disease.

Authors:  Catherine Sarret; Odile Boespflug-Tanguy; Diana Rodriguez
Journal:  Metab Brain Dis       Date:  2015-11-19       Impact factor: 3.584

9.  N-acetylaspartate supports the energetic demands of developmental myelination via oligodendroglial aspartoacylase.

Authors:  Jeremy S Francis; Ireneusz Wojtas; Vladimir Markov; Steven J Gray; Thomas J McCown; R Jude Samulski; Larissa T Bilaniuk; Dah-Jyuu Wang; Darryl C De Vivo; Christopher G Janson; Paola Leone
Journal:  Neurobiol Dis       Date:  2016-10-04       Impact factor: 5.996

Review 10.  Canavan Disease as a Model for Gene Therapy-Mediated Myelin Repair.

Authors:  Anoushka Lotun; Dominic J Gessler; Guangping Gao
Journal:  Front Cell Neurosci       Date:  2021-04-23       Impact factor: 6.147
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