Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice
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- 作者：Owen M. Peters
- 关键词：Amyotrophic lateral sclerosis (ALS) ; frontotemporal dementia (FTD) ; C9ORF72 ; transgenic mice ; RNA foci ; RAN translation ; repeat expansions ; neurodegeneration ; microRNA
- 刊名：Neuron
- 出版年：2015
- 出版时间：2 December 2015
- 年：2015
- 卷：88
- 期：5
- 页码：902-909
- 全文大小：1760 K
文摘
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- <p id="p0010">Mouse model expressing ALS/FTD associated C9ORF72 hexanucleotide repeat expansionp>
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- <p id="p0015">Formation of sense and antisense C9ORF72 RNA transcript focip>
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- <p id="p0020">Dipeptide repeat proteins produced by repeat-associated non-ATG translationp>
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- <p id="p0025">Formation of cytoplasmic inclusions containing dipeptide repeat proteinsp>