Altered phosphorylation and intracellular distribution of a (CUG)n triplet repeat RNA-binding protein in patients with myotonic dystrophy and in myotonin protein kinase knockout mice - Wikidata - awgldk - TriplyDB

Altered phosphorylation and intracellular distribution of a (CUG)n triplet repeat RNA-binding protein in patients with myotonic dystrophy and in myotonin protein kinase knockout mice

Altered phosphorylation and intracellular distribution of a (CUG)n triplet repeat RNA-binding protein in patients with myotonic dystrophy and in myotonin protein kinase knockout mice

http://www.wikidata.org/entity/Q24654728

about

A family of human RNA-binding proteins related to the Drosophila Bruno translational regulator The CELF family of RNA binding proteins is implicated in cell-specific and developmentally regulated alternative splicing Divergent mitochondrial and endoplasmic reticulum association of DMPK splice isoforms depends on unique sequence arrangements in tail anchors.Epidermal growth factor receptor stimulation activates the RNA binding protein CUG-BP1 and increases expression of C/EBPbeta-LIP in mammary epithelial cells CUG-BP binds to RNA substrates and recruits PARN deadenylase Molecular basis for impaired muscle differentiation in myotonic dystrophy In vivo co-localisation of MBNL protein with DMPK expanded-repeat transcripts ETR-3 and CELF4 protein domains required for RNA binding and splicing activity in vivo HnRNP H inhibits nuclear export of mRNA containing expanded CUG repeats and a distal branch point sequence.Feedback Regulation of Kinase Signaling Pathways by AREs and GREs The importance of CELF control: molecular and biological roles of the CUG-BP, Elav-like family of RNA-binding proteins Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis Evolutionary conservation and expression of human RNA-binding proteins and their role in human genetic disease Late-Onset Alzheimer's Disease Genes and the Potentially Implicated Pathways RNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1 c-Jun ARE targets mRNA deadenylation by an EDEN-BP (embryo deadenylation element-binding protein)-dependent pathway TIA-1 and TIAR activate splicing of alternative exons with weak 5' splice sites followed by a U-rich stretch on their own pre-mRNAs Systematic analysis of cis-elements in unstable mRNAs demonstrates that CUGBP1 is a key regulator of mRNA decay in muscle cells Hypogonadism Associated with Cyp19a1 (Aromatase) Posttranscriptional Upregulation in Celf1 Knockout Mice.The unfoldomics decade: an update on intrinsically disordered proteins.Understanding protein non-folding.Repeat length and RNA expression level are not primary determinants in CUG expansion toxicity in Drosophila models Unfoldomics of human diseases: linking protein intrinsic disorder with diseases Muscle-specific splicing of a heterologous exon mediated by a single muscle-specific splicing enhancer from the cardiac troponin T gene.DMPK dosage alterations result in atrioventricular conduction abnormalities in a mouse myotonic dystrophy model.Myotonic dystrophies 1 and 2: complex diseases with complex mechanisms.Expression of a dominant negative CELF protein in vivo leads to altered muscle organization, fiber size, and subtype.Myotonic dystrophy: the role of RNA CUG triplet repeats.The structural basis of myotonic dystrophy from the crystal structure of CUG repeats Structural genomics analysis of alternative splicing and application to isoform structure modeling.Does (CUG)n repeat in DMPK mRNA 'paint' chromosome 19 to suppress distant genes to create the diverse phenotype of myotonic dystrophy?: A new hypothesis of long-range cis autosomal inactivation.An analysis of the sequence requirements of EDEN-BP for specific RNA binding.Smaug/SAMD4A restores translational activity of CUGBP1 and suppresses CUG-induced myopathy.Molecular biology of heart disease.CUG-binding protein represses translation of p27Kip1 mRNA through its internal ribosomal entry site Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation The perinucleolar compartment and transcription.Bcl-x pre-mRNA splicing regulates brain injury after neonatal hypoxia-ischemia.CELFish ways to modulate mRNA decay.The cell biology of disease: cellular and molecular mechanisms underlying muscular dystrophy.

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P2860

Altered phosphorylation and intracellular distribution of a (CUG)n triplet repeat RNA-binding protein in patients with myotonic dystrophy and in myotonin protein kinase knockout mice

http://www.wikidata.org/entity/Q24654728

description

1997 nî lūn-bûn

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1997 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած

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1997 թվականի նոյեմբերին հրատարակված գիտական հոդված

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1997年の論文

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1997年論文

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1997年論文

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1997年論文

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1997年論文

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1997年論文

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Altered phosphorylation and in ...... n protein kinase knockout mice

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Proceedings of the National Academy of Sciences of the United States of America

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Altered phosphorylation and in ...... n protein kinase knockout mice

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C T Caskey

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J W Miller

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L T Timchenko

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N A Timchenko

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R Roberts

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S Reddy

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P2860

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

Myotonic dystrophy mutation: an unstable CTG repeat in the 3' untranslated region of the gene

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

Cloning of the essential myotonic dystrophy region and mapping of the putative defect.

The correlation of age of onset with CTG trinucleotide repeat amplification in myotonic dystrophy.

Molecular basis of myotonic dystrophy: expansion of a trinucleotide (CTG) repeat at the 3' end of a transcript encoding a protein kinase family member.

Full-length myotonin protein kinase (72 kDa) displays serine kinase activity

Correlation between CTG trinucleotide repeat length and frequency of severe congenital myotonic dystrophy.

An unstable triplet repeat in a gene related to myotonic muscular dystrophy.

Decreased expression of myotonin-protein kinase messenger RNA and protein in adult form of myotonic dystrophy.

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13221-13226

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24

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94

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Maurice S. Swanson

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13857769

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9371827

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phosphorylation

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24290

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