TOM COOPER to Myotonic Dystrophy
This is a "connection" page, showing publications TOM COOPER has written about Myotonic Dystrophy.
Connection Strength
12.522
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MBNL overexpression rescues cardiac phenotypes in a myotonic dystrophy type 1 heart mouse model. J Clin Invest. 2025 Feb 11; 135(7).
Score: 0.813
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Small Molecule Screening Identifies HSP90 as a Modifier of RNA Foci in Myotonic Dystrophy Type 1. Mol Cell Biol. 2025; 45(6):225-237.
Score: 0.795
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Rescue of Scn5a mis-splicing does not improve the structural and functional heart defects of a DM1 heart mouse model. Hum Mol Genet. 2024 10 07; 33(20):1789-1799.
Score: 0.794
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The role of Limch1 alternative splicing in skeletal muscle function. Life Sci Alliance. 2023 06; 6(6).
Score: 0.714
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Alternative splicing mediates the compensatory upregulation of MBNL2 upon MBNL1 loss-of-function. Nucleic Acids Res. 2023 02 22; 51(3):1245-1259.
Score: 0.709
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Clinical and Molecular Insights into Gastrointestinal Dysfunction in Myotonic Dystrophy Types 1 & 2. Int J Mol Sci. 2022 Nov 26; 23(23).
Score: 0.698
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Increased nuclear but not cytoplasmic activities of CELF1 protein leads to muscle wasting. Hum Mol Genet. 2020 06 27; 29(10):1729-1744.
Score: 0.590
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Endurance exercise leads to beneficial molecular and physiological effects in a mouse model of myotonic dystrophy type 1. Muscle Nerve. 2019 12; 60(6):779-789.
Score: 0.563
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Mechanisms of skeletal muscle wasting in a mouse model for myotonic dystrophy type 1. Hum Mol Genet. 2018 08 15; 27(16):2789-2804.
Score: 0.518
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Roles for RNA-binding proteins in development and disease. Brain Res. 2016 09 15; 1647:1-8.
Score: 0.438
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The Mef2 transcription network is disrupted in myotonic dystrophy heart tissue, dramatically altering miRNA and mRNA expression. Cell Rep. 2014 Jan 30; 6(2):336-45.
Score: 0.377
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Reexpression of pyruvate kinase M2 in type 1 myofibers correlates with altered glucose metabolism in myotonic dystrophy. Proc Natl Acad Sci U S A. 2013 Aug 13; 110(33):13570-5.
Score: 0.365
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Antisense oligonucleotides: rising stars in eliminating RNA toxicity in myotonic dystrophy. Hum Gene Ther. 2013 May; 24(5):499-507.
Score: 0.353
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RNase H-mediated degradation of toxic RNA in myotonic dystrophy type 1. Proc Natl Acad Sci U S A. 2012 Mar 13; 109(11):4221-6.
Score: 0.331
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CUGBP1 overexpression in mouse skeletal muscle reproduces features of myotonic dystrophy type 1. Hum Mol Genet. 2010 Sep 15; 19(18):3614-22.
Score: 0.295
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Heart-specific overexpression of CUGBP1 reproduces functional and molecular abnormalities of myotonic dystrophy type 1. Hum Mol Genet. 2010 Mar 15; 19(6):1066-75.
Score: 0.285
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Pathogenic mechanisms of myotonic dystrophy. Biochem Soc Trans. 2009 Dec; 37(Pt 6):1281-6.
Score: 0.284
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PKC inhibition ameliorates the cardiac phenotype in a mouse model of myotonic dystrophy type 1. J Clin Invest. 2009 Dec; 119(12):3797-806.
Score: 0.282
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Chemical reversal of the RNA gain of function in myotonic dystrophy. Proc Natl Acad Sci U S A. 2009 Nov 03; 106(44):18433-4.
Score: 0.282
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Molecular biology. Neutralizing toxic RNA. Science. 2009 Jul 17; 325(5938):272-3.
Score: 0.276
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Expanded CTG repeats within the DMPK 3' UTR causes severe skeletal muscle wasting in an inducible mouse model for myotonic dystrophy. Proc Natl Acad Sci U S A. 2008 Feb 19; 105(7):2646-51.
Score: 0.250
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Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation. Mol Cell. 2007 Oct 12; 28(1):68-78.
Score: 0.245
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Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy. J Clin Invest. 2007 Oct; 117(10):2802-11.
Score: 0.244
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Alternative splicing in disease. Adv Exp Med Biol. 2007; 623:212-23.
Score: 0.232
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A reversal of misfortune for myotonic dystrophy? N Engl J Med. 2006 Oct 26; 355(17):1825-7.
Score: 0.229
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Misregulation of alternative splicing causes pathogenesis in myotonic dystrophy. Prog Mol Subcell Biol. 2006; 44:133-59.
Score: 0.216
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Colocalization of muscleblind with RNA foci is separable from mis-regulation of alternative splicing in myotonic dystrophy. J Cell Sci. 2005 Jul 01; 118(Pt 13):2923-33.
Score: 0.208
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Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophy. Hum Mol Genet. 2005 Jun 01; 14(11):1539-47.
Score: 0.206
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Loss of the muscle-specific chloride channel in type 1 myotonic dystrophy due to misregulated alternative splicing. Mol Cell. 2002 Jul; 10(1):45-53.
Score: 0.170
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Aberrant regulation of insulin receptor alternative splicing is associated with insulin resistance in myotonic dystrophy. Nat Genet. 2001 Sep; 29(1):40-7.
Score: 0.160
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Disruption of splicing regulated by a CUG-binding protein in myotonic dystrophy. Science. 1998 May 01; 280(5364):737-41.
Score: 0.127
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RNA-binding proteins in microsatellite expansion disorders: mediators of RNA toxicity. Brain Res. 2012 Jun 26; 1462:100-11.
Score: 0.083
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Alternative splicing dysregulation secondary to skeletal muscle regeneration. Ann Neurol. 2011 Apr; 69(4):681-90.
Score: 0.077
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Overexpression of MBNL1 fetal isoforms and modified splicing of Tau in the DM1 brain: two individual consequences of CUG trinucleotide repeats. Exp Neurol. 2008 Apr; 210(2):467-78.
Score: 0.062
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MBNL1 and CUGBP1 modify expanded CUG-induced toxicity in a Drosophila model of myotonic dystrophy type 1. Hum Mol Genet. 2006 Jul 01; 15(13):2138-45.
Score: 0.056
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Insulin receptor splicing alteration in myotonic dystrophy type 2. Am J Hum Genet. 2004 Jun; 74(6):1309-13.
Score: 0.048
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Pre-mRNA splicing and human disease. Genes Dev. 2003 Feb 15; 17(4):419-37.
Score: 0.044
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Myotonic dystrophy: discussion of molecular basis. Adv Exp Med Biol. 2002; 516:27-45.
Score: 0.041
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RNA processing and human disease. Cell Mol Life Sci. 2000 Feb; 57(2):235-49.
Score: 0.036
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Antagonistic regulation of mRNA expression and splicing by CELF and MBNL proteins. Genome Res. 2015 Jun; 25(6):858-71.
Score: 0.026