Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation
about
Use of model systems to understand the etiology of fragile X-associated primary ovarian insufficiency (FXPOI)MBNL and CELF proteins regulate alternative splicing of the skeletal muscle chloride channel CLCN1Muscleblind-like 2-mediated alternative splicing in the developing brain and dysregulation in myotonic dystrophyThe pathobiology of splicingElevation of RNA-binding protein CUGBP1 is an early event in an inducible heart-specific mouse model of myotonic dystrophyGenes associated with Alzheimer's disease: an overview and current statusEpigenetics and Triplet-Repeat Neurological DiseasesFeedback Regulation of Kinase Signaling Pathways by AREs and GREsCongenital and childhood myotonic dystrophy: Current aspects of disease and future directionsThe importance of CELF control: molecular and biological roles of the CUG-BP, Elav-like family of RNA-binding proteinsRNA-Binding Proteins: Splicing Factors and DiseaseThe multiple molecular facets of fragile X-associated tremor/ataxia syndromeConverging mechanisms in ALS and FTD: disrupted RNA and protein homeostasisLate-Onset Alzheimer's Disease Genes and the Potentially Implicated PathwaysStructural Insights into RNA Recognition by the Alternate-Splicing Regulator CUG-Binding Protein 1Alternative splicing of myomesin 1 gene is aberrantly regulated in myotonic dystrophy type 1Systematic analysis of cis-elements in unstable mRNAs demonstrates that CUGBP1 is a key regulator of mRNA decay in muscle cellsA postnatal switch of CELF and MBNL proteins reprograms alternative splicing in the developing heartRNA and diseaseDisease Phenotypes in a Mouse Model of RNA Toxicity Are Independent of Protein Kinase Cα and Protein Kinase CβEvaluating the effects of CELF1 deficiency in a mouse model of RNA toxicityTailoring of membrane proteins by alternative splicing of pre-mRNA.The RNA-binding protein Staufen1 is increased in DM1 skeletal muscle and promotes alternative pre-mRNA splicingTherapeutics development in myotonic dystrophy type 1.New function for the RNA helicase p68/DDX5 as a modifier of MBNL1 activity on expanded CUG repeatsThe alternative heart: impact of alternative splicing in heart disease.Expanded CTG repeats within the DMPK 3' UTR causes severe skeletal muscle wasting in an inducible mouse model for myotonic dystrophyPKC inhibition ameliorates the cardiac phenotype in a mouse model of myotonic dystrophy type 1.CUGBP1 overexpression in mouse skeletal muscle reproduces features of myotonic dystrophy type 1.Abnormal splicing switch of DMD's penultimate exon compromises muscle fibre maintenance in myotonic dystrophy.Repression of nuclear CELF activity can rescue CELF-regulated alternative splicing defects in skeletal muscle models of myotonic dystrophyTargeting toxic RNAs that cause myotonic dystrophy type 1 (DM1) with a bisamidinium inhibitor.Heart-specific overexpression of CUGBP1 reproduces functional and molecular abnormalities of myotonic dystrophy type 1.Targeting DMPK with Antisense Oligonucleotide Improves Muscle Strength in Myotonic Dystrophy Type 1 MiceMicroRNAs coordinate an alternative splicing network during mouse postnatal heart developmentAberrant alternative splicing and extracellular matrix gene expression in mouse models of myotonic dystrophy.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.Splicing biomarkers of disease severity in myotonic dystrophyIn vivo discovery of a peptide that prevents CUG-RNA hairpin formation and reverses RNA toxicity in myotonic dystrophy models
P2860
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P2860
Increased steady-state levels of CUGBP1 in myotonic dystrophy 1 are due to PKC-mediated hyperphosphorylation
description
2007 nî lūn-bûn
@nan
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
2007年论文
@zh
2007年论文
@zh-cn
name
Increased steady-state levels ...... -mediated hyperphosphorylation
@ast
Increased steady-state levels ...... -mediated hyperphosphorylation
@en
type
label
Increased steady-state levels ...... -mediated hyperphosphorylation
@ast
Increased steady-state levels ...... -mediated hyperphosphorylation
@en
prefLabel
Increased steady-state levels ...... -mediated hyperphosphorylation
@ast
Increased steady-state levels ...... -mediated hyperphosphorylation
@en
P2860
P1433
P1476
Increased steady-state levels ...... -mediated hyperphosphorylation
@en
P2093
N Muge Kuyumcu-Martinez
Thomas A Cooper
P2860
P356
10.1016/J.MOLCEL.2007.07.027
P577
2007-10-01T00:00:00Z