Published in Gene Expr Patterns on August 01, 2003
Transcriptome-wide regulation of pre-mRNA splicing and mRNA localization by muscleblind proteins. Cell (2012) 3.20
MBNL binds similar RNA structures in the CUG repeats of myotonic dystrophy and its pre-mRNA substrate cardiac troponin T. RNA (2007) 2.58
Pentamidine reverses the splicing defects associated with myotonic dystrophy. Proc Natl Acad Sci U S A (2009) 2.21
Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic. Cell Rep (2013) 1.96
RNA protein interaction in neurons. Annu Rev Neurosci (2013) 1.31
Genetic and chemical modifiers of a CUG toxicity model in Drosophila. PLoS One (2008) 1.25
Misregulation of alternative splicing causes pathogenesis in myotonic dystrophy. Prog Mol Subcell Biol (2006) 1.16
Prediction of clustered RNA-binding protein motif sites in the mammalian genome. Nucleic Acids Res (2013) 1.05
MBNL proteins and their target RNAs, interaction and splicing regulation. Nucleic Acids Res (2014) 1.00
Novel mutation and three other sequence variants segregating with phenotype at keratoconus 13q32 susceptibility locus. Eur J Hum Genet (2011) 0.98
MBNL1-RNA recognition: contributions of MBNL1 sequence and RNA conformation. Chembiochem (2011) 0.96
Muscleblind-like 1 (Mbnl1) regulates pre-mRNA alternative splicing during terminal erythropoiesis. Blood (2014) 0.95
Progressive impairment of muscle regeneration in muscleblind-like 3 isoform knockout mice. Hum Mol Genet (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
Epigenetic changes and non-coding expanded repeats. Neurobiol Dis (2010) 0.91
Caenorhabditis elegans Muscleblind homolog mbl-1 functions in neurons to regulate synapse formation. Neural Dev (2012) 0.91
Expression pattern of muscleblind-like proteins differs in differentiating myoblasts. Biochem Biophys Res Commun (2007) 0.86
RNA binding proteins in the regulation of heart development. Int J Biochem Cell Biol (2013) 0.84
Transcription factors CTCF and Pax6 are segregated to different cell types during retinal cell differentiation. Dev Dyn (2008) 0.83
Loss of muscleblind-like 1 promotes invasive mesenchyme formation in endocardial cushions by stimulating autocrine TGFβ3. BMC Dev Biol (2012) 0.83
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
Transcriptional changes and developmental abnormalities in a zebrafish model of myotonic dystrophy type 1. Dis Model Mech (2013) 0.82
Alternative splicing of the neurofibromatosis type 1 pre-mRNA is regulated by the muscleblind-like proteins and the CUG-BP and ELAV-like factors. BMC Mol Biol (2012) 0.81
Loss of muscleblind-like 1 results in cardiac pathology and persistence of embryonic splice isoforms. Sci Rep (2015) 0.81
Abnormal splicing switch of DMD's penultimate exon compromises muscle fibre maintenance in myotonic dystrophy. Nat Commun (2015) 0.81
Molecular mechanisms in DM1 - a focus on foci. Nucleic Acids Res (2015) 0.81
miR-30-5p Regulates Muscle Differentiation and Alternative Splicing of Muscle-Related Genes by Targeting MBNL. Int J Mol Sci (2016) 0.79
Roles for RNA-binding proteins in development and disease. Brain Res (2016) 0.79
Two enhancers control transcription of Drosophila muscleblind in the embryonic somatic musculature and in the central nervous system. PLoS One (2014) 0.79
Sense and Antisense DMPK RNA Foci Accumulate in DM1 Tissues during Development. PLoS One (2015) 0.78
Mechanistic determinants of MBNL activity. Nucleic Acids Res (2016) 0.77
RNA-binding protein misregulation in microsatellite expansion disorders. Adv Exp Med Biol (2014) 0.77
Muscleblind-like 1 suppresses breast cancer metastatic colonization and stabilizes metastasis suppressor transcripts. Genes Dev (2016) 0.77
Identification of Targets of CUG-BP, Elav-Like Family Member 1 (CELF1) Regulation in Embryonic Heart Muscle. PLoS One (2016) 0.76
Disease Phenotypes in a Mouse Model of RNA Toxicity Are Independent of Protein Kinase Cα and Protein Kinase Cβ. PLoS One (2016) 0.75
Dysregulation of mRNA Localization and Translation in Genetic Disease. J Neurosci (2016) 0.75
Myotonic dystrophy: approach to therapy. Curr Opin Genet Dev (2017) 0.75
MBNL1-mediated regulation of differentiation RNAs promotes myofibroblast transformation and the fibrotic response. Nat Commun (2015) 0.75
Conservation of context-dependent splicing activity in distant Muscleblind homologs. Nucleic Acids Res (2016) 0.75
Monoclonal antibodies against Muscleblind-like 3, a protein with punctate nuclear localization. Hybridoma (Larchmt) (2011) 0.75
Autoregulation of MBNL1 function by exon 1 exclusion from MBNL1 transcript. Nucleic Acids Res (2016) 0.75
Paradoxical overexpression of MBNL2 in hepatocellular carcinoma inhibits tumor growth and invasion. Oncotarget (2016) 0.75
A muscleblind knockout model for myotonic dystrophy. Science (2003) 5.93
Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy. Hum Mol Genet (2006) 4.35
Muscleblind proteins regulate alternative splicing. EMBO J (2004) 4.01
Non-ATG-initiated translation directed by microsatellite expansions. Proc Natl Acad Sci U S A (2010) 3.58
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
RNA gain-of-function in spinocerebellar ataxia type 8. PLoS Genet (2009) 2.45
Colocalization of muscleblind with RNA foci is separable from mis-regulation of alternative splicing in myotonic dystrophy. J Cell Sci (2005) 2.31
ALK5- and TGFBR2-independent role of ALK1 in the pathogenesis of hereditary hemorrhagic telangiectasia type 2. Blood (2007) 2.17
RNA localization in yeast: moving towards a mechanism. Biol Cell (2005) 2.15
Real-time imaging of de novo arteriovenous malformation in a mouse model of hereditary hemorrhagic telangiectasia. J Clin Invest (2009) 2.13
Dual requirement for yeast hnRNP Nab2p in mRNA poly(A) tail length control and nuclear export. EMBO J (2002) 2.07
Small molecule regulators of protein arginine methyltransferases. J Biol Chem (2004) 1.99
Nitric oxide cytoskeletal-induced alterations reverse the endothelial progenitor cell migratory defect associated with diabetes. Diabetes (2006) 1.96
Dynamic balance between activation and repression regulates pre-mRNA alternative splicing during heart development. Dev Dyn (2005) 1.95
Arterial endothelium-specific activin receptor-like kinase 1 expression suggests its role in arterialization and vascular remodeling. Circ Res (2003) 1.87
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
Resveratrol improves insulin signaling in a tissue-specific manner under insulin-resistant conditions only: in vitro and in vivo experiments in rodents. Metabolism (2011) 1.76
Genetic ablation of the BMPR2 gene in pulmonary endothelium is sufficient to predispose to pulmonary arterial hypertension. Circulation (2008) 1.72
Fractalkine and CX 3 CR1 regulate hippocampal neurogenesis in adult and aged rats. Neurobiol Aging (2009) 1.72
Minimal homozygous endothelial deletion of Eng with VEGF stimulation is sufficient to cause cerebrovascular dysplasia in the adult mouse. Cerebrovasc Dis (2012) 1.68
Adeno-associated virus type 2 VP2 capsid protein is nonessential and can tolerate large peptide insertions at its N terminus. J Virol (2004) 1.67
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
Arteriovenous malformation in the adult mouse brain resembling the human disease. Ann Neurol (2011) 1.55
A mouse model for hereditary hemorrhagic telangiectasia (HHT) type 2. Hum Mol Genet (2003) 1.38
Partners in crime: bidirectional transcription in unstable microsatellite disease. Hum Mol Genet (2010) 1.37
Subfertility, uterine hypoplasia, and partial progesterone resistance in mice lacking the Kruppel-like factor 9/basic transcription element-binding protein-1 (Bteb1) gene. J Biol Chem (2004) 1.33
Splicing biomarkers of disease severity in myotonic dystrophy. Ann Neurol (2013) 1.22
Recruitment of transcription complexes to the beta-globin gene locus in vivo and in vitro. J Biol Chem (2004) 1.21
Myosin light chain phosphorylation is critical for adaptation to cardiac stress. Circulation (2012) 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
Transforming growth factor-beta1 increases CXCR4 expression, stromal-derived factor-1alpha-stimulated signalling and human immunodeficiency virus-1 entry in human monocyte-derived macrophages. Immunology (2005) 1.15
Nonoverlapping expression patterns of ALK1 and ALK5 reveal distinct roles of each receptor in vascular development. Lab Invest (2006) 1.14
Evolutionarily conserved mammalian adenine nucleotide translocase 4 is essential for spermatogenesis. J Biol Chem (2007) 1.11
Vitreous levels of vascular endothelial growth factor and stromal-derived factor 1 in patients with diabetic retinopathy and cystoid macular edema before and after intraocular injection of triamcinolone. Arch Ophthalmol (2004) 1.11
Reduced mural cell coverage and impaired vessel integrity after angiogenic stimulation in the Alk1-deficient brain. Arterioscler Thromb Vasc Biol (2012) 1.11
Toxic RNA in the nucleus: unstable microsatellite expression in neuromuscular disease. Prog Mol Subcell Biol (2004) 1.09
B-cell translocation gene 2 (Btg2) regulates vertebral patterning by modulating bone morphogenetic protein/smad signaling. Mol Cell Biol (2004) 1.08
Developments and general features of national health insurance in Korea. Soc Work Public Health (2010) 1.08
Dysregulation of intestinal crypt cell proliferation and villus cell migration in mice lacking Kruppel-like factor 9. Am J Physiol Gastrointest Liver Physiol (2007) 1.07
Prediction of clustered RNA-binding protein motif sites in the mammalian genome. Nucleic Acids Res (2013) 1.05
Muscleblind-like 1 knockout mice reveal novel splicing defects in the myotonic dystrophy brain. PLoS One (2012) 1.05
Conditional deletion of Jak2 reveals an essential role in hematopoiesis throughout mouse ontogeny: implications for Jak2 inhibition in humans. PLoS One (2013) 1.03
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
TGF-beta1 upregulates CX3CR1 expression and inhibits fractalkine-stimulated signaling in rat microglia. J Neuroimmunol (2002) 1.01
Isolation of a regulatory region of activin receptor-like kinase 1 gene sufficient for arterial endothelium-specific expression. Circ Res (2004) 1.01
A murine model for human sepiapterin-reductase deficiency. Am J Hum Genet (2006) 1.00
Chemokine receptor CXCR3 promotes growth of glioma. Carcinogenesis (2010) 0.99
Muscleblind isoforms are functionally distinct and regulate alpha-actinin splicing. Differentiation (2007) 0.99
Adenine nucleotide translocase 4 deficiency leads to early meiotic arrest of murine male germ cells. Reproduction (2009) 0.99
Impaired terminal differentiation of hippocampal granule neurons and defective contextual memory in PC3/Tis21 knockout mice. PLoS One (2009) 0.98
The facial motor nucleus transcriptional program in response to peripheral nerve injury identifies Hn1 as a regeneration-associated gene. J Neurosci Res (2005) 0.98
TIS21 negatively regulates hepatocarcinogenesis by disruption of cyclin B1-Forkhead box M1 regulation loop. Hepatology (2008) 0.98
CX3CL1 and CX3CR1 in the GL261 murine model of glioma: CX3CR1 deficiency does not impact tumor growth or infiltration of microglia and lymphocytes. J Neuroimmunol (2008) 0.97
MicroRNAs in mammalian development and tumorigenesis. Birth Defects Res C Embryo Today (2006) 0.97
TGF-β signaling in endothelial cells, but not neuroepithelial cells, is essential for cerebral vascular development. Lab Invest (2011) 0.96
Bone morphogenetic protein-9 inhibits lymphatic vessel formation via activin receptor-like kinase 1 during development and cancer progression. Proc Natl Acad Sci U S A (2013) 0.96
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
Identification of chemical structure and free radical scavenging activity of diphlorethohydroxycarmalol isolated from a brown alga, Ishige okamurae. J Microbiol Biotechnol (2008) 0.95
Tnk1/Kos1 knockout mice develop spontaneous tumors. Cancer Res (2008) 0.93
Progressive impairment of muscle regeneration in muscleblind-like 3 isoform knockout mice. Hum Mol Genet (2013) 0.92
Growth differentiation factor 11 signaling controls retinoic acid activity for axial vertebral development. Dev Biol (2010) 0.89
Pinin modulates expression of an intestinal homeobox gene, Cdx2, and plays an essential role for small intestinal morphogenesis. Dev Biol (2010) 0.89
A model of GAG/MIP-2/CXCR2 interfaces and its functional effects. Biochemistry (2012) 0.89
SMAD1 deficiency in either endothelial or smooth muscle cells can predispose mice to pulmonary hypertension. Hypertension (2013) 0.88
Generation of mice with a conditional and reporter allele for Tmem100. Genesis (2010) 0.88
Development of an optical fiber sensor for angular displacement measurements. Biomed Mater Eng (2014) 0.87
Application for the wearable heart activity monitoring system: analysis of the autonomic function of HRV. Conf Proc IEEE Eng Med Biol Soc (2008) 0.86
Portable activity monitoring system for temporal parameters of gait cycles. J Med Syst (2009) 0.85
Role of Pinin in neural crest, dorsal dermis, and axial skeleton development and its involvement in the regulation of Tcf/Lef activity in mice. Dev Dyn (2007) 0.84
In situ hybridization analysis of chemokines and chemokine receptors in the central nervous system. Methods (2003) 0.84
Enhanced responses to angiogenic cues underlie the pathogenesis of hereditary hemorrhagic telangiectasia 2. PLoS One (2013) 0.84
Myotonic dystrophy CTG expansion affects synaptic vesicle proteins, neurotransmission and mouse behaviour. Brain (2013) 0.84
CXCR3 antagonism of SDF-1(5-67) restores trabecular function and prevents retinal neurodegeneration in a rat model of ocular hypertension. PLoS One (2012) 0.83
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
TIS21/(BTG2) negatively regulates estradiol-stimulated expansion of hematopoietic stem cells by derepressing Akt phosphorylation and inhibiting mTOR signal transduction. Stem Cells (2008) 0.82
Spectral imaging reveals microvessel physiology and function from anastomoses to thromboses. J Biomed Opt (2010) 0.82
Promotion effect of norgalanthamine, a component of Crinum asiaticum, on hair growth. Eur J Dermatol (2009) 0.82
Expression and functional heterogeneity of chemokine receptors CXCR4 and CXCR7 in primary patient-derived glioblastoma cells. PLoS One (2013) 0.82
Vascular smooth muscle Jak2 deletion prevents angiotensin II-mediated neointima formation following injury in mice. J Mol Cell Cardiol (2011) 0.82
Proline 326 in the C terminus of murine CX3CR1 prevents G-protein and phosphatidylinositol 3-kinase-dependent stimulation of Akt and extracellular signal-regulated kinase in Chinese hamster ovary cells. J Pharmacol Exp Ther (2005) 0.82
Generation of activin receptor type IIB isoform-specific hypomorphic alleles. Genesis (2006) 0.81
SnoN facilitates ALK1-Smad1/5 signaling during embryonic angiogenesis. J Cell Biol (2013) 0.81
Protective effect of enzymatic extracts from microalgae against DNA damage induced by H2O2. Mar Biotechnol (NY) (2007) 0.81