Published in Dev Dyn on July 01, 2005
A postnatal switch of CELF and MBNL proteins reprograms alternative splicing in the developing heart. Proc Natl Acad Sci U S A (2008) 3.33
Functional consequences of developmentally regulated alternative splicing. Nat Rev Genet (2011) 3.27
Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation. Mol Cell (2007) 3.26
MBNL binds similar RNA structures in the CUG repeats of myotonic dystrophy and its pre-mRNA substrate cardiac troponin T. RNA (2007) 2.58
Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy. J Clin Invest (2007) 2.14
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) 1.94
Defective splicing, disease and therapy: searching for master checkpoints in exon definition. Nucleic Acids Res (2006) 1.89
Muscleblind-like 2-mediated alternative splicing in the developing brain and dysregulation in myotonic dystrophy. Neuron (2012) 1.78
RNA-dependent integrin alpha3 protein localization regulated by the Muscleblind-like protein MLP1. Nat Cell Biol (2005) 1.57
The importance of CELF control: molecular and biological roles of the CUG-BP, Elav-like family of RNA-binding proteins. Wiley Interdiscip Rev RNA (2011) 1.54
Global regulation of alternative splicing during myogenic differentiation. Nucleic Acids Res (2010) 1.39
Splicing factor and exon profiling across human tissues. Nucleic Acids Res (2010) 1.37
Cardiac tissue-specific repression of CELF activity disrupts alternative splicing and causes cardiomyopathy. Mol Cell Biol (2005) 1.35
Formin follows function: a muscle-specific isoform of FHOD3 is regulated by CK2 phosphorylation and promotes myofibril maintenance. J Cell Biol (2010) 1.24
Neuronal regulation of pre-mRNA splicing by polypyrimidine tract binding proteins, PTBP1 and PTBP2. Crit Rev Biochem Mol Biol (2012) 1.20
Vascular smooth muscle phenotypic diversity and function. Physiol Genomics (2010) 1.18
Posttranscriptional regulation of gene networks by GU-rich elements and CELF proteins. RNA Biol (2008) 1.18
Networking in a global world: establishing functional connections between neural splicing regulators and their target transcripts. RNA (2011) 1.14
Alternative splicing dysregulation secondary to skeletal muscle regeneration. Ann Neurol (2011) 1.09
The impact of alternative splicing in vivo: mouse models show the way. RNA (2007) 1.03
Pathogenic RNAs in microsatellite expansion disease. Neurosci Lett (2009) 1.01
MBNL proteins and their target RNAs, interaction and splicing regulation. Nucleic Acids Res (2014) 1.00
Antagonistic regulation of mRNA expression and splicing by CELF and MBNL proteins. Genome Res (2015) 1.00
The CUGBP2 splicing factor regulates an ensemble of branchpoints from perimeter binding sites with implications for autoregulation. PLoS Genet (2009) 0.99
Drosophila muscleblind is involved in troponin T alternative splicing and apoptosis. PLoS One (2008) 0.99
Flies deficient in Muscleblind protein model features of myotonic dystrophy with altered splice forms of Z-band associated transcripts. Hum Genet (2006) 0.96
Alternative splicing is frequent during early embryonic development in mouse. BMC Genomics (2010) 0.95
Diversity and conservation of CELF1 and CELF2 RNA and protein expression patterns during embryonic development. Dev Dyn (2013) 0.92
Muscleblind-like 1 is a negative regulator of TGF-beta-dependent epithelial-mesenchymal transition of atrioventricular canal endocardial cells. Dev Dyn (2009) 0.91
Imaging the alternative silencing of FGFR2 exon IIIb in vivo. RNA (2006) 0.90
The neurofibromatosis type I pre-mRNA is a novel target of CELF protein-mediated splicing regulation. Nucleic Acids Res (2009) 0.89
Expression of a dominant negative CELF protein in vivo leads to altered muscle organization, fiber size, and subtype. PLoS One (2011) 0.86
Zebrafish deficient for Muscleblind-like 2 exhibit features of myotonic dystrophy. Dis Model Mech (2011) 0.85
RNA binding proteins in the regulation of heart development. Int J Biochem Cell Biol (2013) 0.84
Functional properties and evolutionary splicing constraints on a composite exonic regulatory element of splicing in CFTR exon 12. Nucleic Acids Res (2009) 0.84
Loss of muscleblind-like 1 promotes invasive mesenchyme formation in endocardial cushions by stimulating autocrine TGFβ3. BMC Dev Biol (2012) 0.83
Reduced dosage of pos-1 suppresses Mex mutants and reveals complex interactions among CCCH zinc-finger proteins during Caenorhabditis elegans embryogenesis. Genetics (2006) 0.82
Repression of nuclear CELF activity can rescue CELF-regulated alternative splicing defects in skeletal muscle models of myotonic dystrophy. PLoS Curr (2012) 0.82
The RNA-binding protein Staufen1 impairs myogenic differentiation via a c-myc-dependent mechanism. Mol Biol Cell (2014) 0.81
Molecular mechanisms in DM1 - a focus on foci. Nucleic Acids Res (2015) 0.81
Role of myofibril-inducing RNA in cardiac TnT expression in developing Mexican axolotl. Biochem Biophys Res Commun (2007) 0.80
Mechanistic role of a disease-associated genetic variant within the ADAM33 asthma susceptibility gene. BMC Med Genet (2007) 0.78
Sense and Antisense DMPK RNA Foci Accumulate in DM1 Tissues during Development. PLoS One (2015) 0.78
A novel conserved isoform of the ubiquitin ligase UFD2a/UBE4B is expressed exclusively in mature striated muscle cells. PLoS One (2011) 0.77
Gene Expression Analyses during Spontaneous Reversal of Cardiomyopathy in Mice with Repressed Nuclear CUG-BP, Elav-Like Family (CELF) Activity in Heart Muscle. PLoS One (2015) 0.77
Identification of transcripts regulated by CUG-BP, Elav-like family member 1 (CELF1) in primary embryonic cardiomyocytes by RNA-seq. Genom Data (2015) 0.77
Celf1 is required for formation of endoderm-derived organs in zebrafish. Int J Mol Sci (2013) 0.76
Muscleblind-like 1 is required for normal heart valve development in vivo. BMC Dev Biol (2015) 0.76
CELF4 Variant and Anthracycline-Related Cardiomyopathy: A Children's Oncology Group Genome-Wide Association Study. J Clin Oncol (2016) 0.76
Identification of Targets of CUG-BP, Elav-Like Family Member 1 (CELF1) Regulation in Embryonic Heart Muscle. PLoS One (2016) 0.76
Transcriptome complexity in cardiac development and diseases--an expanding universe between genome and phenome. Circ J (2014) 0.75
Genome-wide analysis of alternative splicing during human heart development. Sci Rep (2016) 0.75
Neonatal cardiac dysfunction and transcriptome changes caused by the absence of Celf1. Sci Rep (2016) 0.75
Andrea Ladd: Getting to the heart of alternative splicing control. Interview by Ruth Williams. J Cell Biol (2008) 0.75
CUG-BP, Elav-like family member 1 (CELF1) is required for normal myofibrillogenesis, morphogenesis, and contractile function in the embryonic heart. Dev Dyn (2016) 0.75
Structural basis for interaction of the tandem zinc finger domains of human muscleblind with cognate RNA from human cardiac troponin T. Biochemistry (2017) 0.75
Pre-mRNA splicing and human disease. Genes Dev (2003) 9.13
Splicing in disease: disruption of the splicing code and the decoding machinery. Nat Rev Genet (2007) 7.42
A muscleblind knockout model for myotonic dystrophy. Science (2003) 5.93
Finding signals that regulate alternative splicing in the post-genomic era. Genome Biol (2002) 4.73
Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy. Hum Mol Genet (2006) 4.35
RNA-mediated neuromuscular disorders. Annu Rev Neurosci (2006) 4.24
Loss of the muscle-specific chloride channel in type 1 myotonic dystrophy due to misregulated alternative splicing. Mol Cell (2002) 4.05
Muscleblind proteins regulate alternative splicing. EMBO J (2004) 4.01
Expression of 24,426 human alternative splicing events and predicted cis regulation in 48 tissues and cell lines. Nat Genet (2008) 3.78
Non-ATG-initiated translation directed by microsatellite expansions. Proc Natl Acad Sci U S A (2010) 3.58
The pINDUCER lentiviral toolkit for inducible RNA interference in vitro and in vivo. Proc Natl Acad Sci U S A (2011) 3.40
A postnatal switch of CELF and MBNL proteins reprograms alternative splicing in the developing heart. Proc Natl Acad Sci U S A (2008) 3.33
Functional consequences of developmentally regulated alternative splicing. Nat Rev Genet (2011) 3.27
Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation. Mol Cell (2007) 3.26
Myotonic dystrophy type 1 is associated with nuclear foci of mutant RNA, sequestration of muscleblind proteins and deregulated alternative splicing in neurons. Hum Mol Genet (2004) 3.21
Reversal of RNA missplicing and myotonia after muscleblind overexpression in a mouse poly(CUG) model for myotonic dystrophy. Proc Natl Acad Sci U S A (2006) 3.09
Aberrant alternative splicing and extracellular matrix gene expression in mouse models of myotonic dystrophy. Nat Struct Mol Biol (2010) 3.07
Muscleblind-like 1 interacts with RNA hairpins in splicing target and pathogenic RNAs. Nucleic Acids Res (2007) 2.95
Essential role for Dicer during skeletal muscle development. Dev Biol (2007) 2.76
Correction of ClC-1 splicing eliminates chloride channelopathy and myotonia in mouse models of myotonic dystrophy. J Clin Invest (2007) 2.68
Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophy. Hum Mol Genet (2005) 2.59
The pathobiology of splicing. J Pathol (2010) 2.52
RNA gain-of-function in spinocerebellar ataxia type 8. PLoS Genet (2009) 2.45
Pathogenic mechanisms of myotonic dystrophy. Biochem Soc Trans (2009) 2.32
Colocalization of muscleblind with RNA foci is separable from mis-regulation of alternative splicing in myotonic dystrophy. J Cell Sci (2005) 2.31
Dynamic antagonism between ETR-3 and PTB regulates cell type-specific alternative splicing. Mol Cell (2002) 2.27
Pre-mRNA splicing in disease and therapeutics. Trends Mol Med (2012) 2.23
Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy. J Clin Invest (2007) 2.14
Dual requirement for yeast hnRNP Nab2p in mRNA poly(A) tail length control and nuclear export. EMBO J (2002) 2.07
Identification of putative new splicing targets for ETR-3 using sequences identified by systematic evolution of ligands by exponential enrichment. Mol Cell Biol (2005) 2.03
Small molecule regulators of protein arginine methyltransferases. J Biol Chem (2004) 1.99
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) 1.94
Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy. J Gen Physiol (2006) 1.83
Transcriptional and post-transcriptional impact of toxic RNA in myotonic dystrophy. Hum Mol Genet (2009) 1.80
Muscleblind-like 2-mediated alternative splicing in the developing brain and dysregulation in myotonic dystrophy. Neuron (2012) 1.78
MBNL1 and CUGBP1 modify expanded CUG-induced toxicity in a Drosophila model of myotonic dystrophy type 1. Hum Mol Genet (2006) 1.77
Mechanisms of RNA-mediated disease. J Biol Chem (2008) 1.59
Nuclear RNA foci in the heart in myotonic dystrophy. Circ Res (2005) 1.57
Developmental expression of mouse muscleblind genes Mbnl1, Mbnl2 and Mbnl3. Gene Expr Patterns (2003) 1.49
Antagonistic regulation of alpha-actinin alternative splicing by CELF proteins and polypyrimidine tract binding protein. RNA (2003) 1.47
CUGBP1 overexpression in mouse skeletal muscle reproduces features of myotonic dystrophy type 1. Hum Mol Genet (2010) 1.44
CELF6, a member of the CELF family of RNA-binding proteins, regulates muscle-specific splicing enhancer-dependent alternative splicing. J Biol Chem (2004) 1.43
RNase H-mediated degradation of toxic RNA in myotonic dystrophy type 1. Proc Natl Acad Sci U S A (2012) 1.40
Global regulation of alternative splicing during myogenic differentiation. Nucleic Acids Res (2010) 1.39
Partners in crime: bidirectional transcription in unstable microsatellite disease. Hum Mol Genet (2010) 1.37
Heart-specific overexpression of CUGBP1 reproduces functional and molecular abnormalities of myotonic dystrophy type 1. Hum Mol Genet (2010) 1.35
Cardiac tissue-specific repression of CELF activity disrupts alternative splicing and causes cardiomyopathy. Mol Cell Biol (2005) 1.35
Minigene reporter for identification and analysis of cis elements and trans factors affecting pre-mRNA splicing. Biotechniques (2006) 1.30
A bichromatic fluorescent reporter for cell-based screens of alternative splicing. Nucleic Acids Res (2006) 1.26
Alternative splicing in disease. Adv Exp Med Biol (2007) 1.22
MicroRNAs coordinate an alternative splicing network during mouse postnatal heart development. Genes Dev (2010) 1.22
Splicing biomarkers of disease severity in myotonic dystrophy. Ann Neurol (2013) 1.22
Identification of MBNL1 and MBNL3 domains required for splicing activation and repression. Nucleic Acids Res (2010) 1.20
Myosin light chain phosphorylation is critical for adaptation to cardiac stress. Circulation (2012) 1.19
PKC inhibition ameliorates the cardiac phenotype in a mouse model of myotonic dystrophy type 1. J Clin Invest (2009) 1.19
Compound loss of muscleblind-like function in myotonic dystrophy. EMBO Mol Med (2013) 1.19
Developments in RNA splicing and disease. Cold Spring Harb Perspect Biol (2011) 1.18
Misregulation of alternative splicing causes pathogenesis in myotonic dystrophy. Prog Mol Subcell Biol (2006) 1.16
ETR-3 and CELF4 protein domains required for RNA binding and splicing activity in vivo. Nucleic Acids Res (2004) 1.16
Identification of CELF splicing activation and repression domains in vivo. Nucleic Acids Res (2005) 1.12
Multiple domains control the subcellular localization and activity of ETR-3, a regulator of nuclear and cytoplasmic RNA processing events. J Cell Sci (2004) 1.11
RNA-binding proteins in microsatellite expansion disorders: mediators of RNA toxicity. Brain Res (2012) 1.11
Alternative splicing dysregulation secondary to skeletal muscle regeneration. Ann Neurol (2011) 1.09
Toxic RNA in the nucleus: unstable microsatellite expression in neuromuscular disease. Prog Mol Subcell Biol (2004) 1.09
Myotonic dystrophy mouse models: towards rational therapy development. Trends Mol Med (2011) 1.07
Muscleblind-like 1 knockout mice reveal novel splicing defects in the myotonic dystrophy brain. PLoS One (2012) 1.05
Prediction of clustered RNA-binding protein motif sites in the mammalian genome. Nucleic Acids Res (2013) 1.05
Identification of NH...N hydrogen bonds by magic angle spinning solid state NMR in a double-stranded RNA associated with myotonic dystrophy. Nucleic Acids Res (2004) 1.01
Pathogenic RNAs in microsatellite expansion disease. Neurosci Lett (2009) 1.01
Muscleblind isoforms are functionally distinct and regulate alpha-actinin splicing. Differentiation (2007) 0.99
CUGBP2 directly interacts with U2 17S snRNP components and promotes U2 snRNA binding to cardiac troponin T pre-mRNA. Nucleic Acids Res (2009) 0.97
MicroRNAs in mammalian development and tumorigenesis. Birth Defects Res C Embryo Today (2006) 0.97
Yeast mRNA Poly(A) tail length control can be reconstituted in vitro in the absence of Pab1p-dependent Poly(A) nuclease activity. J Biol Chem (2005) 0.95
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) 0.92
Progressive impairment of muscle regeneration in muscleblind-like 3 isoform knockout mice. Hum Mol Genet (2013) 0.92
Expression, localization and tau exon 10 splicing activity of the brain RNA-binding protein TNRC4. Hum Mol Genet (2007) 0.90
Myotonic dystrophy: discussion of molecular basis. Adv Exp Med Biol (2002) 0.85
Muscleblind-like 1 activates insulin receptor exon 11 inclusion by enhancing U2AF65 binding and splicing of the upstream intron. Nucleic Acids Res (2013) 0.84
Myotonic dystrophy CTG expansion affects synaptic vesicle proteins, neurotransmission and mouse behaviour. Brain (2013) 0.84
Generation of neural cells from DM1 induced pluripotent stem cells as cellular model for the study of central nervous system neuropathogenesis. Cell Reprogram (2013) 0.82
Antisense oligonucleotides: rising stars in eliminating RNA toxicity in myotonic dystrophy. Hum Gene Ther (2013) 0.80
Constraints on the structure of (CUG)97 RNA from magic-angle-spinning solid-state NMR spectroscopy. Angew Chem Int Ed Engl (2006) 0.78
(1)H, (15)N and (13)C chemical shift assignments of RNA repeats binding protein -- CUGBP1ab. J Biomol NMR (2004) 0.78
Micromanaging alternative splicing during muscle differentiation. Dev Cell (2007) 0.78
RNA-binding proteins in heart development. Adv Exp Med Biol (2014) 0.78
RNA-binding protein misregulation in microsatellite expansion disorders. Adv Exp Med Biol (2014) 0.77
Global insights into alternative polyadenylation regulation. RNA Biol (2015) 0.76
In Brief: (mis)splicing in disease. J Pathol (2014) 0.75
Regulation of chloride ion conductance during skeletal muscle development and in disease. Focus on "Chloride channelopathy in myotonic dystrophy resulting from loss of posttranscriptional regulation for CLCN1". Am J Physiol Cell Physiol (2007) 0.75
Regulating mRNA complexity in the mammalian brain. Nat Genet (2011) 0.75
International Anesthesia Research Society Annual Meeting: then & now. Anesth Analg (2012) 0.75
Double the trouble: bidirectional expression of the SCA8 CAG/CTG expansion mutation - evidence for RNA and protein gain of function effects. Rinsho Shinkeigaku (2010) 0.75
BNA(NC) gapmers revert splicing and reduce RNA foci with low toxicity in myotonic dystrophy cells. ACS Chem Biol (2017) 0.75