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miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis.微小RNA-19、微小RNA-101和微小RNA-130共同调节共济失调蛋白1水平，以潜在地调节脊髓小脑共济失调1型的发病机制。
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2
Beyond the glutamine expansion: influence of posttranslational modifications of ataxin-1 in the pathogenesis of spinocerebellar ataxia type 1.除了谷氨酰胺扩增：ataxin-1的翻译后修饰在1型脊髓小脑共济失调发病机制中的影响
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Purkinje cell ataxin-1 modulates climbing fiber synaptic input in developing and adult mouse cerebellum.浦肯野细胞 ataxin-1 调节发育中和成年小鼠小脑的 climbing fiber 突触输入。
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Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1.聚谷氨酰胺扩增对天然蛋白质复合物的相反作用导致了脊髓小脑共济失调1型（SCA1）。
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本文引用的文献

1
Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1.聚谷氨酰胺扩增对天然蛋白质复合物的相反作用导致了脊髓小脑共济失调1型（SCA1）。
Nature. 2008 Apr 10;452(7188):713-8. doi: 10.1038/nature06731. Epub 2008 Mar 12.
2
Ataxin-1 fusion partners alter polyQ lethality and aggregation.ataxin-1融合伴侣改变多聚谷氨酰胺的致死性和聚集性。
PLoS One. 2007 Oct 10;2(10):e1014. doi: 10.1371/journal.pone.0001014.
3
Cerebellar neurodegeneration in the absence of microRNAs.无微RNA情况下的小脑神经变性
J Exp Med. 2007 Jul 9;204(7):1553-8. doi: 10.1084/jem.20070823. Epub 2007 Jul 2.
4
Trinucleotide repeat disorders.三核苷酸重复序列疾病
Annu Rev Neurosci. 2007;30:575-621. doi: 10.1146/annurev.neuro.29.051605.113042.
5
Combinatorial microRNA target predictions.组合式微小RNA靶标预测
Nat Genet. 2005 May;37(5):495-500. doi: 10.1038/ng1536. Epub 2005 Apr 3.
6
RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia.在脊髓小脑共济失调模型中，RNA干扰抑制多聚谷氨酰胺诱导的神经退行性变。
Nat Med. 2004 Aug;10(8):816-20. doi: 10.1038/nm1076. Epub 2004 Jul 4.
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MicroRNAs: genomics, biogenesis, mechanism, and function.微小RNA：基因组学、生物发生、作用机制及功能
Cell. 2004 Jan 23;116(2):281-97. doi: 10.1016/s0092-8674(04)00045-5.
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Prediction of mammalian microRNA targets.哺乳动物微小RNA靶标的预测
Cell. 2003 Dec 26;115(7):787-98. doi: 10.1016/s0092-8674(03)01018-3.
9
YAC transgenic mice carrying pathological alleles of the MJD1 locus exhibit a mild and slowly progressive cerebellar deficit.携带MJD1基因座病理等位基因的酵母人工染色体转基因小鼠表现出轻度且进展缓慢的小脑缺陷。
Hum Mol Genet. 2002 May 1;11(9):1075-94. doi: 10.1093/hmg/11.9.1075.
10
Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human.ataxin-2的核定位或包涵体形成对于小鼠或人类的脊髓小脑共济失调2型（SCA2）发病机制并非必需。
Nat Genet. 2000 Sep;26(1):44-50. doi: 10.1038/79162.

微小RNA-19、微小RNA-101和微小RNA-130共同调节共济失调蛋白1水平，以潜在地调节脊髓小脑共济失调1型的发病机制。

miR-19, miR-101 and miR-130 co-regulate ATXN1 levels to potentially modulate SCA1 pathogenesis.

作者信息

Lee Yoontae, Samaco Rodney C, Gatchel Jennifer R, Thaller Christina, Orr Harry T, Zoghbi Huda Y

机构信息

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas 77030, USA.

出版信息

Nat Neurosci. 2008 Oct;11(10):1137-9. doi: 10.1038/nn.2183. Epub 2008 Aug 31.

DOI:10.1038/nn.2183
PMID:18758459
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2574629/
Abstract

Spinocerebellar ataxia type 1 is caused by expansion of a translated CAG repeat in ataxin1 (ATXN1). The level of the polyglutamine-expanded protein is one of the factors that contributes to disease severity. Here we found that miR-19, miR-101 and miR-130 co-regulate ataxin1 levels and that their inhibition enhanced the cytotoxicity of polyglutamine-expanded ATXN1 in human cells. We provide a new candidate mechanism for modulating the pathogenesis of neurodegenerative diseases sensitive to protein dosage.

摘要

1型脊髓小脑共济失调是由ataxin1（ATXN1）中一个可翻译的CAG重复序列扩增引起的。聚谷氨酰胺扩增蛋白的水平是导致疾病严重程度的因素之一。在此，我们发现miR-19、miR-101和miR-130共同调节ataxin1的水平，并且抑制它们会增强聚谷氨酰胺扩增的ATXN1在人细胞中的细胞毒性。我们为调节对蛋白质剂量敏感的神经退行性疾病的发病机制提供了一种新的候选机制。