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Items: 1 to 20 of 35

1.

Aberrant alternative splicing and extracellular matrix gene expression in mouse models of myotonic dystrophy.

Du H, Cline MS, Osborne RJ, Tuttle DL, Clark TA, Donohue JP, Hall MP, Shiue L, Swanson MS, Thornton CA, Ares M Jr.

Nat Struct Mol Biol. 2010 Feb;17(2):187-93. doi: 10.1038/nsmb.1720. Epub 2010 Jan 24.

2.

Heart-specific overexpression of CUGBP1 reproduces functional and molecular abnormalities of myotonic dystrophy type 1.

Koshelev M, Sarma S, Price RE, Wehrens XH, Cooper TA.

Hum Mol Genet. 2010 Mar 15;19(6):1066-75. doi: 10.1093/hmg/ddp570. Epub 2010 Jan 5.

3.

PKC inhibition ameliorates the cardiac phenotype in a mouse model of myotonic dystrophy type 1.

Wang GS, Kuyumcu-Martinez MN, Sarma S, Mathur N, Wehrens XH, Cooper TA.

J Clin Invest. 2009 Dec;119(12):3797-806. doi: 10.1172/JCI37976. Epub 2009 Nov 9.

4.

Transcriptional and post-transcriptional impact of toxic RNA in myotonic dystrophy.

Osborne RJ, Lin X, Welle S, Sobczak K, O'Rourke JR, Swanson MS, Thornton CA.

Hum Mol Genet. 2009 Apr 15;18(8):1471-81. doi: 10.1093/hmg/ddp058. Epub 2009 Feb 17.

5.

Satellite cell dysfunction contributes to the progressive muscle atrophy in myotonic dystrophy type 1.

Thornell LE, Lindstöm M, Renault V, Klein A, Mouly V, Ansved T, Butler-Browne G, Furling D.

Neuropathol Appl Neurobiol. 2009 Dec;35(6):603-13. doi: 10.1111/j.1365-2990.2009.01014.x. Epub 2009 Jan 21.

PMID:
19207265
6.

Alternative splicing of RyR1 alters the efficacy of skeletal EC coupling.

Kimura T, Lueck JD, Harvey PJ, Pace SM, Ikemoto N, Casarotto MG, Dirksen RT, Dulhunty AF.

Cell Calcium. 2009 Mar;45(3):264-74. doi: 10.1016/j.ceca.2008.11.005. Epub 2009 Jan 7.

7.

A postnatal switch of CELF and MBNL proteins reprograms alternative splicing in the developing heart.

Kalsotra A, Xiao X, Ward AJ, Castle JC, Johnson JM, Burge CB, Cooper TA.

Proc Natl Acad Sci U S A. 2008 Dec 23;105(51):20333-8. doi: 10.1073/pnas.0809045105. Epub 2008 Dec 15.

8.

Electrocardiographic abnormalities and sudden death in myotonic dystrophy type 1.

Groh WJ, Groh MR, Saha C, Kincaid JC, Simmons Z, Ciafaloni E, Pourmand R, Otten RF, Bhakta D, Nair GV, Marashdeh MM, Zipes DP, Pascuzzi RM.

N Engl J Med. 2008 Jun 19;358(25):2688-97. doi: 10.1056/NEJMoa062800.

9.

Expanded CTG repeats within the DMPK 3' UTR causes severe skeletal muscle wasting in an inducible mouse model for myotonic dystrophy.

Orengo JP, Chambon P, Metzger D, Mosier DR, Snipes GJ, Cooper TA.

Proc Natl Acad Sci U S A. 2008 Feb 19;105(7):2646-51. doi: 10.1073/pnas.0708519105. Epub 2008 Feb 13.

10.

Conserved GU-rich elements mediate mRNA decay by binding to CUG-binding protein 1.

Vlasova IA, Tahoe NM, Fan D, Larsson O, Rattenbacher B, Sternjohn JR, Vasdewani J, Karypis G, Reilly CS, Bitterman PB, Bohjanen PR.

Mol Cell. 2008 Feb 1;29(2):263-70. doi: 10.1016/j.molcel.2007.11.024.

11.

Correction of ClC-1 splicing eliminates chloride channelopathy and myotonia in mouse models of myotonic dystrophy.

Wheeler TM, Lueck JD, Swanson MS, Dirksen RT, Thornton CA.

J Clin Invest. 2007 Dec;117(12):3952-7.

12.
13.

Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy.

Wang GS, Kearney DL, De Biasi M, Taffet G, Cooper TA.

J Clin Invest. 2007 Oct;117(10):2802-11.

14.

Altered mRNA splicing of dystrophin in type 1 myotonic dystrophy.

Nakamori M, Kimura T, Fujimura H, Takahashi MP, Sakoda S.

Muscle Nerve. 2007 Aug;36(2):251-7.

PMID:
17487865
15.

Chloride channelopathy in myotonic dystrophy resulting from loss of posttranscriptional regulation for CLCN1.

Lueck JD, Lungu C, Mankodi A, Osborne RJ, Welle SL, Dirksen RT, Thornton CA.

Am J Physiol Cell Physiol. 2007 Apr;292(4):C1291-7. Epub 2006 Nov 29.

16.

Reversible model of RNA toxicity and cardiac conduction defects in myotonic dystrophy.

Mahadevan MS, Yadava RS, Yu Q, Balijepalli S, Frenzel-McCardell CD, Bourne TD, Phillips LH.

Nat Genet. 2006 Sep;38(9):1066-70. Epub 2006 Jul 30.

17.

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
18.

Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy.

Lin X, Miller JW, Mankodi A, Kanadia RN, Yuan Y, Moxley RT, Swanson MS, Thornton CA.

Hum Mol Genet. 2006 Jul 1;15(13):2087-97. Epub 2006 May 22.

PMID:
16717059
19.

Defective neuromuscular synaptogenesis in mice expressing constitutively active ErbB2 in skeletal muscle fibers.

Ponomareva ON, Ma H, Vock VM, Ellerton EL, Moody SE, Dakour R, Chodosh LA, Rimer M.

Mol Cell Neurosci. 2006 Feb;31(2):334-45. Epub 2005 Nov 8.

PMID:
16278083
20.

Altered mRNA splicing of the skeletal muscle ryanodine receptor and sarcoplasmic/endoplasmic reticulum Ca2+-ATPase in myotonic dystrophy type 1.

Kimura T, Nakamori M, Lueck JD, Pouliquin P, Aoike F, Fujimura H, Dirksen RT, Takahashi MP, Dulhunty AF, Sakoda S.

Hum Mol Genet. 2005 Aug 1;14(15):2189-200. Epub 2005 Jun 22.

PMID:
15972723

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