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MicroRNA-277 modulates the neurodegeneration caused by Fragile X premutation rCGG repeats.

Tan H, Poidevin M, Li H, Chen D, Jin P.

PLoS Genet. 2012;8(5):e1002681. doi: 10.1371/journal.pgen.1002681. Epub 2012 May 3.


Pur alpha binds to rCGG repeats and modulates repeat-mediated neurodegeneration in a Drosophila model of fragile X tremor/ataxia syndrome.

Jin P, Duan R, Qurashi A, Qin Y, Tian D, Rosser TC, Liu H, Feng Y, Warren ST.

Neuron. 2007 Aug 16;55(4):556-64.


Retrotransposon activation contributes to fragile X premutation rCGG-mediated neurodegeneration.

Tan H, Qurashi A, Poidevin M, Nelson DL, Li H, Jin P.

Hum Mol Genet. 2012 Jan 1;21(1):57-65. doi: 10.1093/hmg/ddr437. Epub 2011 Sep 22.


Nuclear accumulation of stress response mRNAs contributes to the neurodegeneration caused by Fragile X premutation rCGG repeats.

Qurashi A, Li W, Zhou JY, Peng J, Jin P.

PLoS Genet. 2011 Jun;7(6):e1002102. doi: 10.1371/journal.pgen.1002102. Epub 2011 Jun 2.


RNA-binding proteins hnRNP A2/B1 and CUGBP1 suppress fragile X CGG premutation repeat-induced neurodegeneration in a Drosophila model of FXTAS.

Sofola OA, Jin P, Qin Y, Duan R, Liu H, de Haro M, Nelson DL, Botas J.

Neuron. 2007 Aug 16;55(4):565-71.


Chemical screen reveals small molecules suppressing fragile X premutation rCGG repeat-mediated neurodegeneration in Drosophila.

Qurashi A, Liu H, Ray L, Nelson DL, Duan R, Jin P.

Hum Mol Genet. 2012 May 1;21(9):2068-75. doi: 10.1093/hmg/dds024. Epub 2012 Feb 1.


RNA-mediated neurodegeneration caused by the fragile X premutation rCGG repeats in Drosophila.

Jin P, Zarnescu DC, Zhang F, Pearson CE, Lucchesi JC, Moses K, Warren ST.

Neuron. 2003 Aug 28;39(5):739-47.


CGG repeats in RNA modulate expression of TDP-43 in mouse and fly models of fragile X tremor ataxia syndrome.

Galloway JN, Shaw C, Yu P, Parghi D, Poidevin M, Jin P, Nelson DL.

Hum Mol Genet. 2014 Nov 15;23(22):5906-15. doi: 10.1093/hmg/ddu314. Epub 2014 Jun 30.


RNA-mediated neurodegeneration in fragile X-associated tremor/ataxia syndrome.

Li Y, Jin P.

Brain Res. 2012 Jun 26;1462:112-7. doi: 10.1016/j.brainres.2012.02.057. Epub 2012 Mar 9. Review.


TDP-43 suppresses CGG repeat-induced neurotoxicity through interactions with HnRNP A2/B1.

He F, Krans A, Freibaum BD, Taylor JP, Todd PK.

Hum Mol Genet. 2014 Oct 1;23(19):5036-51. doi: 10.1093/hmg/ddu216. Epub 2014 May 8.


Activation of mTOR ameliorates fragile X premutation rCGG repeat-mediated neurodegeneration.

Lin Y, Tang C, He H, Duan R.

PLoS One. 2013 Apr 23;8(4):e62572. doi: 10.1371/journal.pone.0062572. Print 2013.


Ectopic expression of CGG containing mRNA is neurotoxic in mammals.

Hashem V, Galloway JN, Mori M, Willemsen R, Oostra BA, Paylor R, Nelson DL.

Hum Mol Genet. 2009 Jul 1;18(13):2443-51. doi: 10.1093/hmg/ddp182. Epub 2009 Apr 18.


Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome.

Todd PK, Oh SY, Krans A, Pandey UB, Di Prospero NA, Min KT, Taylor JP, Paulson HL.

PLoS Genet. 2010 Dec 9;6(12):e1001240. doi: 10.1371/journal.pgen.1001240.


Argonaute-2-dependent rescue of a Drosophila model of FXTAS by FRAXE premutation repeat.

Sofola OA, Jin P, Botas J, Nelson DL.

Hum Mol Genet. 2007 Oct 1;16(19):2326-32. Epub 2007 Jul 17.


Fragile X-associated tremor/ataxia syndrome: influence of the FMR1 gene on motor fiber tracts in males with normal and premutation alleles.

Wang JY, Hessl D, Schneider A, Tassone F, Hagerman RJ, Rivera SM.

JAMA Neurol. 2013 Aug;70(8):1022-9. doi: 10.1001/jamaneurol.2013.2934.


RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome.

Oh SY, He F, Krans A, Frazer M, Taylor JP, Paulson HL, Todd PK.

Hum Mol Genet. 2015 Aug 1;24(15):4317-26. doi: 10.1093/hmg/ddv165. Epub 2015 May 7.


Expression of the GABAergic system in animal models for fragile X syndrome and fragile X associated tremor/ataxia syndrome (FXTAS).

D'Hulst C, Heulens I, Brouwer JR, Willemsen R, De Geest N, Reeve SP, De Deyn PP, Hassan BA, Kooy RF.

Brain Res. 2009 Feb 9;1253:176-83. doi: 10.1016/j.brainres.2008.11.075. Epub 2008 Dec 3.


Volumetric brain changes in females with fragile X-associated tremor/ataxia syndrome (FXTAS).

Adams JS, Adams PE, Nguyen D, Brunberg JA, Tassone F, Zhang W, Koldewyn K, Rivera SM, Grigsby J, Zhang L, DeCarli C, Hagerman PJ, Hagerman RJ.

Neurology. 2007 Aug 28;69(9):851-9.


Fragile X-associated tremor/ataxia syndrome.

Hagerman PJ, Hagerman RJ.

Ann N Y Acad Sci. 2015 Mar;1338:58-70. doi: 10.1111/nyas.12693. Epub 2015 Jan 26. Review.


The fragile X-associated tremor ataxia syndrome.

Tassone F, Hagerman R.

Results Probl Cell Differ. 2012;54:337-57. doi: 10.1007/978-3-642-21649-7_18.


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