Research Topics
Genomes and Genes | T A CooperSummaryAffiliation: Baylor College of Medicine Country: USA Publications
| Collaborators
|
Detail Information
Publications
RNA and diseaseThomas A Cooper
Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
Cell 136:777-93. 2009..The discovery of disease-causing mutations in RNAs is yielding a wealth of new therapeutic targets, and the growing understanding of RNA biology and chemistry is providing new RNA-based tools for developing therapeutics...- Muscle-specific splicing of a heterologous exon mediated by a single muscle-specific splicing enhancer from the cardiac troponin T geneT A Cooper
Departments of Pathology and Cell Biology, Baylor College of Medicine, Houston, Texas 77030, USA
Mol Cell Biol 18:4519-25. 1998..MSE2 and MSE4 each contain a novel sequence motif that is found adjacent to a number of alternative exons that undergo regulated splicing in striated muscle, suggesting a common role for this element in muscle-specific regulation... 
Highlights of alternative splicing regulation session: yes, no, maybe--a history of paradigm shiftsT A Cooper
Departments of Pathology and Molecular and Cellular Biology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
Sci STKE 2001:pe35. 2001..The importance of properly regulated splicing is emphasized by examples of disease pathologies in which alternative splicing is aberrant...
Finding signals that regulate alternative splicing in the post-genomic eraAndrea N Ladd
Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
Genome Biol 3:reviews0008. 2002..Here, we discuss the regulatory elements that direct alternative splicing and how genome-wide analyses can aid in their identification...- Aberrant regulation of insulin receptor alternative splicing is associated with insulin resistance in myotonic dystrophyR S Savkur
Department of Pathology, Baylor College of Medicine, One Baylor Plaza, Houston, Texas, USA
Nat Genet 29:40-7. 2001..These results support a model in which increased expression of a splicing regulator contributes to insulin resistance in DM1 by affecting IR alternative splicing... - The CELF family of RNA binding proteins is implicated in cell-specific and developmentally regulated alternative splicingA N Ladd
Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
Mol Cell Biol 21:1285-96. 2001..We propose that ETR-3 is a major regulator of cTNT alternative splicing and that the CELF family plays an important regulatory role in cell-specific alternative splicing during normal development and disease... - A subset of SR proteins activates splicing of the cardiac troponin T alternative exon by direct interactions with an exonic enhancerJ Ramchatesingh
Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
Mol Cell Biol 15:4898-907. 1995..Independent regulation of the levels of SR proteins may, therefore, contribute to the developmental regulation of exon inclusion... - Identification of a new class of exonic splicing enhancers by in vivo selectionL R Coulter
Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
Mol Cell Biol 17:2143-50. 1997..We suggest the possibility that the dsx enhancer is a member of a previously unrecognized family of ACEs... - Insulin receptor splicing alteration in myotonic dystrophy type 2R S Savkur
Department of Pathology, Baylor University, Houston, TX, USA
Am J Hum Genet 74:1309-13. 2004..We now demonstrate that comparable splicing abnormalities occur in DM2 muscle prior to the development of muscle histopathology, thus demonstrating an early pathogenic effect of RNA expansions... - Use of minigene systems to dissect alternative splicing elementsThomas A Cooper
Department of Pathology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
Methods 37:331-40. 2005.... - Minigene reporter for identification and analysis of cis elements and trans factors affecting pre-mRNA splicingGopal Singh
Baylor College of Medicine, Houston, TX, USA
Biotechniques 41:177-81. 2006.... - Antagonistic regulation of alpha-actinin alternative splicing by CELF proteins and polypyrimidine tract binding proteinNatalia Gromak
Department of Biochemistry, University of Cambridge, Cambridge CB2 1GA, UK
RNA 9:443-56. 2003.... - A reversal of misfortune for myotonic dystrophy?Thomas A Cooper
Department of Pathology, Baylor College of Medicine, Houston, USA
N Engl J Med 355:1825-7. 2006 - Misregulation of alternative splicing causes pathogenesis in myotonic dystrophyN Muge Kuyumcu-Martinez
Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
Prog Mol Subcell Biol 44:133-59. 2006..Here we review the proposed mechanisms for the toxic effects of the expanded repeats and discuss the molecular mechanisms of splicing misregulation and disease pathogenesis... - A bichromatic fluorescent reporter for cell-based screens of alternative splicingJames P Orengo
Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
Nucleic Acids Res 34:e148. 2006.... - Micromanaging alternative splicing during muscle differentiationChristopher S Bland
Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
Dev Cell 12:171-2. 2007..The results illustrate how two modes of posttranscriptional regulation combine to direct skeletal muscle development... - Expression, localization and tau exon 10 splicing activity of the brain RNA-binding protein TNRC4J Paul Chapple
MRC Centre for Neurodegeneration Research, Institute of Psychiatry, King s College London, De Crespigny Park, London SE5 8AF, UK
Hum Mol Genet 16:2760-9. 2007..This study represents the first characterization of TNRC4 and provides further insight into the mechanisms of brain-specific alternative splicing and their possible pathological implications... - Splicing in disease: disruption of the splicing code and the decoding machineryGuey Shin Wang
Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
Nat Rev Genet 8:749-61. 2007..An unexpectedly large fraction of exonic mutations exhibit a primary pathogenic effect on splicing. Furthermore, normal genetic variation significantly contributes to disease severity and susceptibility by affecting splicing efficiency... - Elevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophyGuey Shin Wang
Department of Pathology, Baylor College of Medicine, Houston, Texas, USA
J Clin Invest 117:2802-11. 2007..These results indicate that CUGBP1 upregulation is an early and primary response to expression of CUG repeat RNA... - Alternative splicing in diseaseJames P Orengo
Department of Pathology, Baylor College of Medicine, Houston, Texas, USA
Adv Exp Med Biol 623:212-23. 2007.... - Loss of the muscle-specific chloride channel in type 1 myotonic dystrophy due to misregulated alternative splicingNicolas Charlet-B
Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
Mol Cell 10:45-53. 2002..We propose that disruption of alternative splicing regulation causes a predominant pathological feature of DM1... - Cardiac tissue-specific repression of CELF activity disrupts alternative splicing and causes cardiomyopathyAndrea N Ladd
Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
Mol Cell Biol 25:6267-78. 2005..We conclude that CELF protein activity is required for normal alternative splicing in the heart in vivo and that normal CELF-mediated alternative splicing regulation is in turn required for normal cardiac function... - Dynamic antagonism between ETR-3 and PTB regulates cell type-specific alternative splicingNicolas Charlet-B
Department of Pathology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
Mol Cell 9:649-58. 2002..We conclude that cell-specific regulation results from the dominance of one among actively competing regulatory states rather than modulation of a nonregulated default state... - Pre-mRNA splicing and human diseaseNuno André Faustino
Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
Genes Dev 17:419-37. 2003 - CELF6, a member of the CELF family of RNA-binding proteins, regulates muscle-specific splicing enhancer-dependent alternative splicingAndrea N Ladd
Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
J Biol Chem 279:17756-64. 2004..These results place CELF6 in a functional subfamily of CELF proteins that includes CELFs 3, 4, and 5. CELF6 also promotes skipping of exon 11 of insulin receptor, a known target of CELF activity that is expressed in kidney... - Multiple domains control the subcellular localization and activity of ETR-3, a regulator of nuclear and cytoplasmic RNA processing eventsAndrea N Ladd
Department of Pathology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
J Cell Sci 117:3519-29. 2004..This is the first characterization of protein domains involved in mediating the subcellular localization and splicing activity of a member of the CELF family of RNA processing regulators... - Muscleblind proteins regulate alternative splicingThai H Ho
Department of Molecular and Cellular Biology, Baylor College of Medicine, Houston, TX 77030, USA
EMBO J 23:3103-12. 2004..The results are consistent with a mechanism for DM pathogenesis in which expanded repeats cause a loss of MBNL and/or gain of CELF activities, leading to misregulation of alternative splicing of specific pre-mRNA targets... - Alternative splicing regulation impacts heart developmentThomas A Cooper
Department of Pathology, Baylor College of Medicine, Houston, TX 77030, USA
Cell 120:1-2. 2005..In this issue of Cell, Xu et al. (2005) demonstrate that a heart-specific knockout of one SR protein, ASF/SF2, produces cardiomyopathy and misregulation of specific alternative splicing events during early postnatal development... - Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophyThai H Ho
Department of Pathology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
Hum Mol Genet 14:1539-47. 2005..The results are consistent with a mechanism for DM pathogenesis in which expanded repeats result in increased CUG-BP1 activity and/or other CELF family members and have trans-dominant effects on specific pre-mRNA targets... - Identification of CELF splicing activation and repression domains in vivoJin Han
Department of Pathology, Baylor College of Medicine Houston, TX 77030, USA
Nucleic Acids Res 33:2769-80. 2005..These results provide a foundation for identifying CELF-interacting proteins involved in activated and/or repressed splicing... - Colocalization of muscleblind with RNA foci is separable from mis-regulation of alternative splicing in myotonic dystrophyThai H Ho
Department of Molecular and Cellular Biology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
J Cell Sci 118:2923-33. 2005..We also find an immobile fraction of GFP-MBNL1 in DM1 fibroblasts and a similar rapid exchange in endogenous CUG RNA foci. Therefore, formation of RNA foci and disruption of MBNL1-regulated splicing are separable events... - Dynamic balance between activation and repression regulates pre-mRNA alternative splicing during heart developmentAndrea N Ladd
Department of Pathology, Baylor College of Medicine, Houston, Texas 77030, USA
Dev Dyn 233:783-93. 2005..ETR-3 and CUG-BP proteins are also down-regulated in other tissues during development, suggesting that CELF proteins play a broad role in developmental splicing regulation...