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Items: 1 to 20 of 23

1.

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.

2.

FMR1 RNA within the intranuclear inclusions of fragile X-associated tremor/ataxia syndrome (FXTAS).

Tassone F, Iwahashi C, Hagerman PJ.

RNA Biol. 2004 Jul;1(2):103-5. Epub 2004 Jul 17.

PMID:
17179750
3.

Altered expressions of the noncoding hsromega gene enhances poly-Q-induced neurotoxicity in Drosophila.

Sengupta S, Lakhotia SC.

RNA Biol. 2006 Jan-Mar;3(1):28-35. Epub 2006 Feb 1.

PMID:
17114940
4.

RNA-mediated neuromuscular disorders.

Ranum LP, Cooper TA.

Annu Rev Neurosci. 2006;29:259-77. Review.

PMID:
16776586
5.

MBNL1 and CUGBP1 modify expanded CUG-induced toxicity in a Drosophila model of myotonic dystrophy type 1.

de Haro M, Al-Ramahi I, De Gouyon B, Ukani L, Rosa A, Faustino NA, Ashizawa T, Cooper TA, Botas J.

Hum Mol Genet. 2006 Jul 1;15(13):2138-45. Epub 2006 May 24.

PMID:
16723374
6.

Neuropathology of fragile X-associated tremor/ataxia syndrome (FXTAS).

Greco CM, Berman RF, Martin RM, Tassone F, Schwartz PH, Chang A, Trapp BD, Iwahashi C, Brunberg J, Grigsby J, Hessl D, Becker EJ, Papazian J, Leehey MA, Hagerman RJ, Hagerman PJ.

Brain. 2006 Jan;129(Pt 1):243-55. Epub 2005 Dec 5.

PMID:
16332642
7.

Protein composition of the intranuclear inclusions of FXTAS.

Iwahashi CK, Yasui DH, An HJ, Greco CM, Tassone F, Nannen K, Babineau B, Lebrilla CB, Hagerman RJ, Hagerman PJ.

Brain. 2006 Jan;129(Pt 1):256-71. Epub 2005 Oct 24.

PMID:
16246864
8.

Induction of inclusion formation and disruption of lamin A/C structure by premutation CGG-repeat RNA in human cultured neural cells.

Arocena DG, Iwahashi CK, Won N, Beilina A, Ludwig AL, Tassone F, Schwartz PH, Hagerman PJ.

Hum Mol Genet. 2005 Dec 1;14(23):3661-71. Epub 2005 Oct 20.

PMID:
16239243
9.

Myotonic dystrophy: RNA pathogenesis comes into focus.

Ranum LP, Day JW.

Am J Hum Genet. 2004 May;74(5):793-804. Epub 2004 Apr 2. Review.

10.

The fragile-X premutation: a maturing perspective.

Hagerman PJ, Hagerman RJ.

Am J Hum Genet. 2004 May;74(5):805-16. Epub 2004 Mar 29. Review. Erratum in: Am J Hum Genet. 2004 Aug;75(2):352.

11.

Overexpression of CUG triplet repeat-binding protein, CUGBP1, in mice inhibits myogenesis.

Timchenko NA, Patel R, Iakova P, Cai ZJ, Quan L, Timchenko LT.

J Biol Chem. 2004 Mar 26;279(13):13129-39. Epub 2004 Jan 13.

12.

A muscleblind knockout model for myotonic dystrophy.

Kanadia RN, Johnstone KA, Mankodi A, Lungu C, Thornton CA, Esson D, Timmers AM, Hauswirth WW, Swanson MS.

Science. 2003 Dec 12;302(5652):1978-80.

13.

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.

14.

The FMR1 CGG repeat mouse displays ubiquitin-positive intranuclear neuronal inclusions; implications for the cerebellar tremor/ataxia syndrome.

Willemsen R, Hoogeveen-Westerveld M, Reis S, Holstege J, Severijnen LA, Nieuwenhuizen IM, Schrier M, van Unen L, Tassone F, Hoogeveen AT, Hagerman PJ, Mientjes EJ, Oostra BA.

Hum Mol Genet. 2003 May 1;12(9):949-59.

PMID:
12700164
15.

Neuronal intranuclear inclusions in a new cerebellar tremor/ataxia syndrome among fragile X carriers.

Greco CM, Hagerman RJ, Tassone F, Chudley AE, Del Bigio MR, Jacquemont S, Leehey M, Hagerman PJ.

Brain. 2002 Aug;125(Pt 8):1760-71.

PMID:
12135967
16.

The fragile X premutation: into the phenotypic fold.

Hagerman RJ, Hagerman PJ.

Curr Opin Genet Dev. 2002 Jun;12(3):278-83. Review.

PMID:
12076670
17.

Intention tremor, parkinsonism, and generalized brain atrophy in male carriers of fragile X.

Hagerman RJ, Leehey M, Heinrichs W, Tassone F, Wilson R, Hills J, Grigsby J, Gage B, Hagerman PJ.

Neurology. 2001 Jul 10;57(1):127-30.

PMID:
11445641
18.

RNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1.

Timchenko NA, Cai ZJ, Welm AL, Reddy S, Ashizawa T, Timchenko LT.

J Biol Chem. 2001 Mar 16;276(11):7820-6. Epub 2000 Dec 21.

19.

The CUG-binding protein binds specifically to UG dinucleotide repeats in a yeast three-hybrid system.

Takahashi N, Sasagawa N, Suzuki K, Ishiura S.

Biochem Biophys Res Commun. 2000 Oct 22;277(2):518-23.

PMID:
11032753
20.

Elevated levels of FMR1 mRNA in carrier males: a new mechanism of involvement in the fragile-X syndrome.

Tassone F, Hagerman RJ, Taylor AK, Gane LW, Godfrey TE, Hagerman PJ.

Am J Hum Genet. 2000 Jan;66(1):6-15.

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