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COOPER, THOMAS
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overview See our lab webpage: https://www.bcm.edu/research/labs/tom-cooper/?PMID=14453 The realization that most human genes generate multiple mRNAs encoding different protein isoforms and variable untranslated regions via alternative splicing and alternative polyadenylation has revealed an extensive degree of regulation that remains to be explored. The Cooper Lab is interested in understanding the mechanisms of this regulation, from how RNA binding proteins regulate splicing of individual pre-mRNAs to the signaling events that coordinate RNA processing networks during development. A second major interest in the lab is the pathogenic mechanism of myotonic dystrophy in which disruption of developmentally regulated RNA processing causes primary features of the disease. Mechanistic understanding of myotonic dystrophy has led to development of therapeutic approaches being testing using mouse models established in the lab.
One or more keywords matched the following items that are connected to COOPER, THOMAS
Item TypeName
Academic Article A 32-nucleotide exon-splicing enhancer regulates usage of competing 5' splice sites in a differential internal exon.
Academic Article A subset of SR proteins activates splicing of the cardiac troponin T alternative exon by direct interactions with an exonic enhancer.
Academic Article Identification of a new class of exonic splicing enhancers by in vivo selection.
Academic Article The regulation of splice-site selection, and its role in human disease.
Academic Article A short sequence within two purine-rich enhancers determines 5' splice site specificity.
Academic Article The cardiac troponin T alternative exon contains a novel purine-rich positive splicing element.
Academic Article In vivo SELEX in vertebrate cells.
Academic Article RNA processing and human disease.
Academic Article Muscle-specific splicing enhancers regulate inclusion of the cardiac troponin T alternative exon in embryonic skeletal muscle.
Academic Article In vitro splicing of cardiac troponin T precursors. Exon mutations disrupt splicing of the upstream intron.
Academic Article Binding of PurH to a muscle-specific splicing enhancer functionally correlates with exon inclusion in vivo.
Academic Article Myotonic dystrophy: discussion of molecular basis.
Academic Article Loss of the muscle-specific chloride channel in type 1 myotonic dystrophy due to misregulated alternative splicing.
Academic Article Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophy.
Academic Article The RNA binding protein YB-1 binds A/C-rich exon enhancers and stimulates splicing of the CD44 alternative exon v4.
Academic Article Colocalization of muscleblind with RNA foci is separable from mis-regulation of alternative splicing in myotonic dystrophy.
Academic Article Pre-mRNA splicing and human disease.
Academic Article Dynamic antagonism between ETR-3 and PTB regulates cell type-specific alternative splicing.
Academic Article Splicing in disease: disruption of the splicing code and the decoding machinery.
Academic Article Identification of putative new splicing targets for ETR-3 using sequences identified by systematic evolution of ligands by exponential enrichment.
Academic Article RNA and disease.
Academic Article Molecular biology. Neutralizing toxic RNA.
Academic Article Identification of CELF splicing activation and repression domains in vivo.
Academic Article Chemical reversal of the RNA gain of function in myotonic dystrophy.
Academic Article Cardiac tissue-specific repression of CELF activity disrupts alternative splicing and causes cardiomyopathy.
Academic Article Multiple domains control the subcellular localization and activity of ETR-3, a regulator of nuclear and cytoplasmic RNA processing events.
Academic Article The pINDUCER lentiviral toolkit for inducible RNA interference in vitro and in vivo.
Academic Article Dynamic balance between activation and repression regulates pre-mRNA alternative splicing during heart development.
Academic Article RNase H-mediated degradation of toxic RNA in myotonic dystrophy type 1.
Academic Article RNA-binding proteins in microsatellite expansion disorders: mediators of RNA toxicity.
Academic Article Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation.
Academic Article Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophy.
Academic Article Alternative splicing in disease.
Academic Article CUGBP2 directly interacts with U2 17S snRNP components and promotes U2 snRNA binding to cardiac troponin T pre-mRNA.
Academic Article PKC inhibition ameliorates the cardiac phenotype in a mouse model of myotonic dystrophy type 1.
Academic Article Pathogenic mechanisms of myotonic dystrophy.
Academic Article The pathobiology of splicing.
Academic Article Heart-specific overexpression of CUGBP1 reproduces functional and molecular abnormalities of myotonic dystrophy type 1.
Academic Article MicroRNAs coordinate an alternative splicing network during mouse postnatal heart development.
Academic Article CUGBP1 overexpression in mouse skeletal muscle reproduces features of myotonic dystrophy type 1.
Academic Article Alternative splicing dysregulation secondary to skeletal muscle regeneration.
Academic Article Global regulation of alternative splicing during myogenic differentiation.
Academic Article Identification of MBNL1 and MBNL3 domains required for splicing activation and repression.
Academic Article Pre-mRNA splicing in disease and therapeutics.
Academic Article Antisense oligonucleotides: rising stars in eliminating RNA toxicity in myotonic dystrophy.
Academic Article Disruption of splicing regulated by a CUG-binding protein in myotonic dystrophy.
Academic Article Muscle-specific splicing of a heterologous exon mediated by a single muscle-specific splicing enhancer from the cardiac troponin T gene.
Academic Article Defining pre-mRNA cis elements that regulate cell-specific splicing.
Academic Article The CELF family of RNA binding proteins is implicated in cell-specific and developmentally regulated alternative splicing.
Academic Article CELF6, a member of the CELF family of RNA-binding proteins, regulates muscle-specific splicing enhancer-dependent alternative splicing.
Academic Article ETR-3 and CELF4 protein domains required for RNA binding and splicing activity in vivo.
Academic Article Muscleblind proteins regulate alternative splicing.
Academic Article Alternative splicing regulation impacts heart development.
Academic Article Use of minigene systems to dissect alternative splicing elements.
Academic Article MBNL1 and CUGBP1 modify expanded CUG-induced toxicity in a Drosophila model of myotonic dystrophy type 1.
Academic Article Minigene reporter for identification and analysis of cis elements and trans factors affecting pre-mRNA splicing.
Academic Article A reversal of misfortune for myotonic dystrophy?
Academic Article Expanded CTG repeats within the DMPK 3' UTR causes severe skeletal muscle wasting in an inducible mouse model for myotonic dystrophy.
Academic Article A postnatal switch of CELF and MBNL proteins reprograms alternative splicing in the developing heart.
Concept Sequence Analysis, RNA
Concept RNA-Binding Protein FUS
Concept RNA Processing, Post-Transcriptional
Concept DEAD-box RNA Helicases
Concept RNA
Concept RNA, Small Nuclear
Concept RNA, Small Interfering
Concept RNA Precursors
Concept RNA Interference
Concept RNA Caps
Concept RNA Splicing
Concept RNA, Untranslated
Concept RNA Probes
Concept Regulatory Sequences, Ribonucleic Acid
Concept RNA-Binding Proteins
Concept RNA Stability
Concept RNA Splice Sites
Concept RNA, Messenger
Academic Article Overexpression of MBNL1 fetal isoforms and modified splicing of Tau in the DM1 brain: two individual consequences of CUG trinucleotide repeats.
Academic Article The relative strengths of SR protein-mediated associations of alternative and constitutive exons can influence alternative splicing.
Academic Article Alternative splicing determines the intracellular localization of the novel nuclear protein Nop30 and its interaction with the splicing factor SRp30c.
Academic Article Insulin receptor splicing alteration in myotonic dystrophy type 2.
Academic Article Muscleblind-like 1 activates insulin receptor exon 11 inclusion by enhancing U2AF65 binding and splicing of the upstream intron.
Academic Article The Mef2 transcription network is disrupted in myotonic dystrophy heart tissue, dramatically altering miRNA and mRNA expression.
Academic Article In Brief: (mis)splicing in disease.
Academic Article Alternative splicing regulates vesicular trafficking genes in cardiomyocytes during postnatal heart development.
Academic Article Rbfox2-coordinated alternative splicing of Mef2d and Rock2 controls myoblast fusion during myogenesis.
Academic Article RNA-binding proteins in heart development.
Academic Article Dynamic analyses of alternative polyadenylation from RNA-seq reveal a 3'-UTR landscape across seven tumour types.
Academic Article Pumilio1 haploinsufficiency leads to SCA1-like neurodegeneration by increasing wild-type Ataxin1 levels.
Academic Article Antagonistic regulation of mRNA expression and splicing by CELF and MBNL proteins.
Academic Article Impaired Mitochondrial Energy Production Causes Light-Induced Photoreceptor Degeneration Independent of Oxidative Stress.
Academic Article The spliceosome is a therapeutic vulnerability in MYC-driven cancer.
Academic Article Roles for RNA-binding proteins in development and disease.
Academic Article The roles of RNA processing in translating genotype to phenotype.
Academic Article Neonatal cardiac dysfunction and transcriptome changes caused by the absence of Celf1.
Academic Article Protein sequestration as a normal function of long noncoding RNAs and a pathogenic mechanism of RNAs containing nucleotide repeat expansions.
Academic Article A Therapeutic Double Whammy: Transcriptional or Post-transcriptional Suppression of Microsatellite Repeat Toxicity by Cas9.
Academic Article Mechanisms of skeletal muscle wasting in a mouse model for myotonic dystrophy type 1.
Academic Article Rbfox-Splicing Factors Maintain Skeletal Muscle Mass by Regulating Calpain3 and Proteostasis.
Academic Article CRISPR -Mediated Expression of the Fetal Scn5a Isoform in Adult Mice Causes Conduction Defects and Arrhythmias.
Grant Mechanisms of Developmentally Regulated Splicing
Grant Molecular Pathogenesis of Myotonic Dystrophy
Grant Deep Sequencing Analysis of mRNA Isoform Expression Changes in Myotonic Dystrophy
Grant Troponin T Alternative Splicing in Embryonic Heart
Concept RNA Splicing Factors
Concept RNA, Long Noncoding
Academic Article Alu insertion variants alter mRNA splicing.
Academic Article Modulation of alternative splicing of trafficking genes by genome editing reveals functional consequences in muscle biology.
Academic Article Increased nuclear but not cytoplasmic activities of CELF1 protein leads to muscle wasting.
Academic Article Overlapping mechanisms of lncRNA and expanded microsatellite RNA.
Academic Article Alternative splicing mediates the compensatory upregulation of MBNL2 upon MBNL1 loss-of-function.
Academic Article Clinical and Molecular Insights into Gastrointestinal Dysfunction in Myotonic Dystrophy Types 1 & 2.
Academic Article The role of Limch1 alternative splicing in skeletal muscle function.
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  • RNA