(Q52654324)

5 May 2018

title

Altered nuclear structure in myotonic dystrophy type 1-derived fibroblasts. (English)

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5 May 2018

Oscar Hernández-Hernández

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object named as

O Hernández-Hernández

2

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Ricardo González-Ramírez

object named as

R González-Ramírez

4

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Jonathan J. Magaña

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author name string

R Rodríguez

1

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5 May 2018

J J Magaña

3

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5 May 2018

E S García-López

5

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5 May 2018

B Cisneros

6

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5 May 2018

publication date

12 October 2014

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5 May 2018

Molecular Biology Reports

published in

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5 May 2018

volume

42

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5 May 2018

issue

2

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5 May 2018

page(s)

479-488

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5 May 2018

99th ENMC international workshop: myotonic dystrophy: present management, future therapy. 9-11 November 2001, Naarden, The Netherlands.

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Expanded CUG repeat RNAs form hairpins that activate the double-stranded RNA-dependent protein kinase PKR

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

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Progressive atrioventricular conduction block in a mouse myotonic dystrophy model.

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Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophy.

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Disruption of splicing regulated by a CUG-binding protein in myotonic dystrophy

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Insulin receptor splicing alteration in myotonic dystrophy type 2.

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RNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1

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Tau exon 2 responsive elements deregulated in myotonic dystrophy type I are proximal to exon 2 and synergistically regulated by MBNL1 and MBNL2.

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Muscleblind-like 1 activates insulin receptor exon 11 inclusion by enhancing U2AF65 binding and splicing of the upstream intron

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Three proteins, MBNL, MBLL and MBXL, co-localize in vivo with nuclear foci of expanded-repeat transcripts in DM1 and DM2 cells.

21 January 2018

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RNA leaching of transcription factors disrupts transcription in myotonic dystrophy

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Muscleblind localizes to nuclear foci of aberrant RNA in myotonic dystrophy types 1 and 2.

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Transcriptionally Repressive Chromatin Remodelling and CpG Methylation in the Presence of Expanded CTG-Repeats at the DM1 Locus

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Distribution of CTG repeats at the DMPK gene in myotonic dystrophy patients and healthy individuals from the Mexican population.

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Biochemical and histochemical analysis of 71 kDa dystrophin isoform (Dp71f) in rat brain

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Dystrophin Dp71 is required for neurite outgrowth in PC12 cells

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Lamin B1 maintains the functional plasticity of nucleoli

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Abnormal nuclear shape and impaired mechanotransduction in emerin-deficient cells

21 January 2018

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Nuclear morphometric features in benign breast tissue and risk of subsequent breast cancer

21 January 2018

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Decreased mechanical stiffness in LMNA-/- cells is caused by defective nucleo-cytoskeletal integrity: implications for the development of laminopathies.

21 January 2018

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Myotonic dystrophy protein kinase is critical for nuclear envelope integrity

21 January 2018

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The yeast lipin Smp2 couples phospholipid biosynthesis to nuclear membrane growth

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Inactivation of the C. elegans lipin homolog leads to ER disorganization and to defects in the breakdown and reassembly of the nuclear envelope

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Aberrant alternative splicing and extracellular matrix gene expression in mouse models of myotonic dystrophy.

21 January 2018

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Farnesylated nuclear proteins Kugelkern and lamin Dm0 affect nuclear morphology by directly interacting with the nuclear membrane.

21 January 2018

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Nuclear shape changes are induced by knockdown of the SWI/SNF ATPase BRG1 and are independent of cytoskeletal connections

21 January 2018

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Separation of silencing from perinuclear anchoring functions in yeast Ku80, Sir4 and Esc1 proteins.

21 January 2018

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"Laminopathies": a wide spectrum of human diseases

21 January 2018

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Inhibition of myogenesis in transgenic mice expressing the human DMPK 3'-UTR.

21 January 2018

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Molecular basis for impaired muscle differentiation in myotonic dystrophy

21 January 2018

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Mutant DMPK 3'-UTR transcripts disrupt C2C12 myogenic differentiation by compromising MyoD.

21 January 2018

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21 January 2018

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Lamin A/C and emerin are critical for skeletal muscle satellite cell differentiation

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The role of nuclear bodies in gene expression and disease

21 January 2018

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CAG expansion induces nucleolar stress in polyglutamine diseases

21 January 2018

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Aberrant regulation of insulin receptor alternative splicing is associated with insulin resistance in myotonic dystrophy

21 January 2018

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12 December 2020

Cis and trans effects of the myotonic dystrophy (DM) mutation in a cell culture model

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12 December 2020

Targeting the nucleolus for cancer intervention

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12 December 2020

Preferential nucleosome assembly at DNA triplet repeats from the myotonic dystrophy gene

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12 December 2020

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12 December 2020

Expanded CTG triplet blocks from the myotonic dystrophy gene create the strongest known natural nucleosome positioning elements

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12 December 2020

The integrity of a lamin-B1-dependent nucleoskeleton is a fundamental determinant of RNA synthesis in human cells

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12 December 2020

Mice lacking the myotonic dystrophy protein kinase develop a late onset progressive myopathy

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12 December 2020

Colocalization of muscleblind with RNA foci is separable from mis-regulation of alternative splicing in myotonic dystrophy

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12 December 2020

10.1007/S11033-014-3791-4

Identifiers

DOI

1 reference

5 May 2018

1 reference

5 May 2018