Expression of RNA CCUG repeats dysregulates translation and degradation of proteins in myotonic dystrophy 2 patients - Wikidata - awgldk - TriplyDB

Expression of RNA CCUG repeats dysregulates translation and degradation of proteins in myotonic dystrophy 2 patients

Expression of RNA CCUG repeats dysregulates translation and degradation of proteins in myotonic dystrophy 2 patients

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ZNF9 activation of IRES-mediated translation of the human ODC mRNA is decreased in myotonic dystrophy type 2 A muscle stem cell for every muscle: variability of satellite cell biology among different muscle groups Muscle wasting in myotonic dystrophies: a model of premature aging Therapeutics development in myotonic dystrophy type 1.Computational investigation of RNA CUG repeats responsible for myotonic dystrophy 1 Myotonic dystrophies 1 and 2: complex diseases with complex mechanisms.Expression profiling of FSHD-1 and FSHD-2 cells during myogenic differentiation evidences common and distinctive gene dysregulation patterns MBNL proteins and their target RNAs, interaction and splicing regulation.Differences in aberrant expression and splicing of sarcomeric proteins in the myotonic dystrophies DM1 and DM2.Mutant (CCTG)n expansion causes abnormal expression of zinc finger protein 9 (ZNF9) in myotonic dystrophy type 2.Expansion of CUG RNA repeats causes stress and inhibition of translation in myotonic dystrophy 1 (DM1) cells Small molecules that target the toxic RNA in myotonic dystrophy type 2.Overexpression of CUGBP1 in skeletal muscle from adult classic myotonic dystrophy type 1 but not from myotonic dystrophy type 2.RNA Foci, CUGBP1, and ZNF9 are the primary targets of the mutant CUG and CCUG repeats expanded in myotonic dystrophies type 1 and type 2 Reduction of the rate of protein translation in patients with myotonic dystrophy 2 Molecular mechanisms of muscle atrophy in myotonic dystrophies.Identifying proteins that bind to specific RNAs - focus on simple repeat expansion diseases.Myotonic dystrophy mouse models: towards rational therapy development.Altered expression and splicing of Ca(2+) metabolism genes in myotonic dystrophies DM1 and DM2.Molecular therapy in myotonic dystrophy: focus on RNA gain-of-function.Repeat expansion diseases: when a good RNA turns bad.The role of CUGBP1 in age-dependent changes of liver functions.Skeletal muscle features in myotonic dystrophy and sarcopenia: do similar nuclear mechanisms lead to skeletal muscle wasting?Clinical aspects, molecular pathomechanisms and management of myotonic dystrophies.RNA Transcription and Maturation in Skeletal Muscle Cells are Similarly Impaired in Myotonic Dystrophy and Sarcopenia: The Ultrastructural Evidence.Muscle weakness in myotonic dystrophy associated with misregulated splicing and altered gating of Ca(V)1.1 calcium channel.(CCUG)n RNA toxicity in a Drosophila model of myotonic dystrophy type 2 (DM2) activates apoptosis.Cultured myoblasts from patients affected by myotonic dystrophy type 2 exhibit senescence-related features: ultrastructural evidence.Myotonic Dystrophy Type 2: An Update on Clinical Aspects, Genetic and Pathomolecular Mechanism.Myotonic dystrophy type 2 and modifier genes: an update on clinical and pathomolecular aspects.Biomolecular diagnosis of myotonic dystrophy type 2: a challenging approach.The origin of genetic instability in CCTG repeats Muscleblind-like1 undergoes ectopic relocation in the nuclei of skeletal muscles in myotonic dystrophy and sarcopenia.A fly model for the CCUG-repeat expansion of myotonic dystrophy type 2 reveals a novel interaction with MBNL1.rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences.RNA processing is altered in skeletal muscle nuclei of patients affected by myotonic dystrophy

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Expression of RNA CCUG repeats dysregulates translation and degradation of proteins in myotonic dystrophy 2 patients

http://www.wikidata.org/entity/Q37278485

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articolo scientifico

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bilimsel makale

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scientific article published on 09 July 2009

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videnskabelig artikel

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vědecký článek

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name

Expression of RNA CCUG repeats ...... myotonic dystrophy 2 patients

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Expression of RNA CCUG repeats ...... myotonic dystrophy 2 patients.

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Expression of RNA CCUG repeats ...... myotonic dystrophy 2 patients

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Expression of RNA CCUG repeats ...... myotonic dystrophy 2 patients.

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prefLabel

Expression of RNA CCUG repeats ...... myotonic dystrophy 2 patients

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Expression of RNA CCUG repeats ...... myotonic dystrophy 2 patients.

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The American Journal of Pathology

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Expression of RNA CCUG repeats ...... myotonic dystrophy 2 patients

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Bingwen Jin

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Claudia Huichalaf

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Elizabeth Salisbury

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Guo-Li Wang

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Lubov T Timchenko

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Mario Sirito

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Nikolai A Timchenko

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Partha Sarkar

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Ralf Krahe

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P2860

Recruitment of human muscleblind proteins to (CUG)(n) expansions associated with myotonic dystrophy

Identification of a (CUG)n triplet repeat RNA-binding protein and its expression in myotonic dystrophy

Failure of MBNL1-dependent post-natal splicing transitions in myotonic dystrophy

Molecular basis for impaired muscle differentiation in myotonic dystrophy

Molecular chaperones in the cytosol: from nascent chain to folded protein

Myotonic dystrophy type 2 caused by a CCTG expansion in intron 1 of ZNF9

Myotonic dystrophy type 2: molecular, diagnostic and clinical spectrum

Novel proteins with binding specificity for DNA CTG repeats and RNA CUG repeats: implications for myotonic dystrophy

Age-specific CUGBP1-eIF2 complex increases translation of CCAAT/enhancer-binding protein beta in old liver

The FMR1 CGG repeat mouse displays ubiquitin-positive intranuclear neuronal inclusions; implications for the cerebellar tremor/ataxia syndrome

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