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

作者: Yoontae Lee , Rodney C Samaco , Jennifer R Gatchel , Christina Thaller , Harry T Orr

DOI: 10.1038/NN.2183

关键词:

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

参考文章(15)
H. Brent Clark, Eric N. Burright, Wael S. Yunis, Seth Larson, Claire Wilcox, Boyd Hartman, Antoni Matilla, Huda Y. Zoghbi, Harry T. Orr, Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations. The Journal of Neuroscience. ,vol. 17, pp. 7385- 7395 ,(1997) , 10.1523/JNEUROSCI.17-19-07385.1997
Duong P. Huynh, Karla Figueroa, Nam Hoang, Stefan-M. Pulst, Nuclear localization or inclusion body formation of ataxin-2 are not necessary for SCA2 pathogenesis in mouse or human. Nature Genetics. ,vol. 26, pp. 44- 50 ,(2000) , 10.1038/79162
Janghoo Lim, Juan Crespo-Barreto, Paymaan Jafar-Nejad, Aaron B. Bowman, Ronald Richman, David E. Hill, Harry T. Orr, Huda Y. Zoghbi, Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1 Nature. ,vol. 452, pp. 713- 718 ,(2008) , 10.1038/NATURE06731
Sandro Banfi, Antonio Servadio, Ming-yi Chung, Thomas J. Kwiatkowski, Alanna E. McCall, Lisa A. Duvick, Ying Shen, Elizabeth J. Roth, Harry T. Orr, H.Y. Zoghbi, Identification and characterization of the gene causing type 1 spinocerebellar ataxia Nature Genetics. ,vol. 7, pp. 513- 520 ,(1994) , 10.1038/NG0894-513
Azra Krek, Dominic Grün, Matthew N Poy, Rachel Wolf, Lauren Rosenberg, Eric J Epstein, Philip MacMenamin, Isabelle da Piedade, Kristin C Gunsalus, Markus Stoffel, Nikolaus Rajewsky, Combinatorial microRNA target predictions Nature Genetics. ,vol. 37, pp. 495- 500 ,(2005) , 10.1038/NG1536
Huda Y. Zoghbi, Harry T. Orr, Spinocerebellar ataxia type 1. Seminars in Cell Biology. ,vol. 6, pp. 29- 35 ,(1995) , 10.1016/1043-4682(95)90012-8
Eric N Burright, H Brent Clark, Antonio Servadio, Toni Matilla, Rodney M Feddersen, Wael S Yunis, Lisa A Duvick, Huda Y Zoghbi, Harry T Orr, SCA1 transgenic mice: A model for neurodegeneration caused by an expanded CAG trinucleotide repeat Cell. ,vol. 82, pp. 937- 948 ,(1995) , 10.1016/0092-8674(95)90273-2
Haibin Xia, Qinwen Mao, Steven L Eliason, Scott Q Harper, Inês H Martins, Harry T Orr, Henry L Paulson, Linda Yang, Robert M Kotin, Beverly L Davidson, RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia Nature Medicine. ,vol. 10, pp. 816- 820 ,(2004) , 10.1038/NM1076