N-acetylaspartate supports the energetic demands of developmental myelination via oligodendroglial aspartoacylase
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- 作者：Jeremy S. Francis ; PhDa ; Ireneusz Wojtas ; PhDa ; Vladimir Markov ; MDa ; Steven J. Gray ; PhDb ; Thomas J. McCown ; PhDc ; R. Jude Samulski ; PhDd ; Larissa T. Bilaniuk ; MDe ; Dah-Jyuu Wang ; PhDe ; Darryl C. De Vivo ; MDf ; Christopher G. Janson ; MDg ; Paola Leone ; PhDa ; leonepa@rowan.edu" class="auth_mail" title="E-mail the corresponding author
- 关键词：N-acetylaspartate ; Aspartoacylase ; Canavan disease ; Myelination ; Energy
- 刊名：Neurobiology of Disease
- 出版年：2016
- 出版时间：December 2016
- 年：2016
- 卷：96
- 期：Complete
- 页码：323-334
- 全文大小：2941 K
文摘
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The loss of aspartoacylase function in Canavan disease is proposed to cause early oxidative stress.
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Metabolic deficits are specific to oligodendrocytes.
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Reconstitution of aspartoacylase in oligodendrocytes promotes energetic integrity.
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Oligodendrocytic energetic integrity in crucial for developmental myelination.
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Phenotypic rescue requires the targeting of oligodendrocytes specifically.