Cargando…

RBFOX1 Cooperates with MBNL1 to Control Splicing in Muscle, Including Events Altered in Myotonic Dystrophy Type 1

With the goal of identifying splicing alterations in myotonic dystrophy 1 (DM1) tissues that may yield insights into targets or mechanisms, we have surveyed mis-splicing events in three systems using a RT-PCR screening and validation platform. First, a transgenic mouse model expressing CUG-repeats i...

Descripción completa

Detalles Bibliográficos
Autores principales:	Klinck, Roscoe, Fourrier, Angélique, Thibault, Philippe, Toutant, Johanne, Durand, Mathieu, Lapointe, Elvy, Caillet-Boudin, Marie-Laure, Sergeant, Nicolas, Gourdon, Geneviève, Meola, Giovanni, Furling, Denis, Puymirat, Jack, Chabot, Benoit
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2014
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4161394/ https://www.ncbi.nlm.nih.gov/pubmed/25211016 http://dx.doi.org/10.1371/journal.pone.0107324

Ejemplares similares

rbFOX1/MBNL1 competition for CCUG RNA repeats binding contributes to myotonic dystrophy type 1/type 2 differences
por: Sellier, Chantal, et al.
Publicado: (2018)

MBNL‐dependent impaired development within the neuromuscular system in myotonic dystrophy type 1
por: Tahraoui‐Bories, Julie, et al.
Publicado: (2023)

Mice lacking MBNL1 and MBNL2 exhibit sudden cardiac death and molecular signatures recapitulating myotonic dystrophy
por: Lee, Kuang-Yung, et al.
Publicado: (2022)

Phenylbutazone induces expression of MBNL1 and suppresses formation of MBNL1-CUG RNA foci in a mouse model of myotonic dystrophy
por: Chen, Guiying, et al.
Publicado: (2016)

Fishing for answers: MBNL2 in myotonic dystrophy
Publicado: (2011)

Loss of MBNL1-mediated retrograde BDNF signaling in the myotonic dystrophy brain
por: Wang, Pei-Ying, et al.
Publicado: (2023)

Brain pathology in myotonic dystrophy: when tauopathy meets spliceopathy and RNAopathy
por: Caillet-Boudin, Marie-Laure, et al.
Publicado: (2014)

RNA/MBNL1-containing foci in myoblast nuclei from patients affected by myotonic dystrophy type 2: an immunocytochemical study
por: Perdoni, F., et al.
Publicado: (2009)

Autistic traits in myotonic dystrophy type 1 due to MBNL inhibition and RNA mis-splicing
por: Sznajder, Lukasz, et al.
Publicado: (2023)

RBFOX1 and RBFOX3 Mutations in Rolandic Epilepsy
por: Lal, Dennis, et al.
Publicado: (2013)

Zebrafish mbnl mutants model physical and molecular phenotypes of myotonic dystrophy
por: Hinman, Melissa N., et al.
Publicado: (2021)

Sustainable recovery of MBNL activity in autoregulatory feedback loop in myotonic dystrophy
por: Rogalska, Zuzanna, et al.
Publicado: (2022)

Calcitriol increases MBNL1 expression and alleviates myotonic dystrophy phenotypes in HSA(LR) mouse models
por: Huang, Kun, et al.
Publicado: (2022)

Peptide-conjugated antimiRs improve myotonic dystrophy type 1 phenotypes by promoting endogenous MBNL1 expression
por: González-Martínez, Irene, et al.
Publicado: (2023)

Autoregulation of MBNL1 function by exon 1 exclusion from MBNL1 transcript
por: Konieczny, Patryk, et al.
Publicado: (2017)

Correction: RBFOX1 and RBFOX3 Mutations in Rolandic Epilepsy
por: Lal, Dennis, et al.
Publicado: (2013)

hnRNP A1/A2 and Sam68 collaborate with SRSF10 to control the alternative splicing response to oxaliplatin-mediated DNA damage
por: Cloutier, Alexandre, et al.
Publicado: (2018)

Electrophysiological basis of cardiac arrhythmia in a mouse model of myotonic dystrophy type 1
por: Ginjupalli, Vamsi Krishna Murthy, et al.
Publicado: (2023)

Myotonic Dystrophies: State of the Art of New Therapeutic Developments for the CNS
por: Gourdon, Genevieve, et al.
Publicado: (2017)

Mbnl1 and Mbnl2 regulate brain structural integrity in mice
por: Sta Maria, Naomi S., et al.
Publicado: (2021)

Dose-Dependent Regulation of Alternative Splicing by MBNL Proteins Reveals Biomarkers for Myotonic Dystrophy
por: Wagner, Stacey D., et al.
Publicado: (2016)

Identification of MBNL1 and MBNL3 domains required for splicing activation and repression
por: Grammatikakis, Ioannis, et al.
Publicado: (2011)

A flow cytometry-based screen identifies MBNL1 modulators that rescue splicing defects in myotonic dystrophy type I
por: Zhang, Fan, et al.
Publicado: (2017)

Proof of concept of peptide-linked blockmiR-induced MBNL functional rescue in myotonic dystrophy type 1 mouse model
por: Overby, Sarah J., et al.
Publicado: (2022)

Antisense oligonucleotides as a potential treatment for brain deficits observed in myotonic dystrophy type 1
por: Ait Benichou, Siham, et al.
Publicado: (2022)

Dysregulation of Circular RNAs in Myotonic Dystrophy Type 1
por: Voellenkle, Christine, et al.
Publicado: (2019)

Synaptic protein dysregulation in myotonic dystrophy type 1: Disease neuropathogenesis beyond missplicing
por: Hernández-Hernández, Oscar, et al.
Publicado: (2013)

SRSF10 Connects DNA Damage to the Alternative Splicing of Transcripts Encoding Apoptosis, Cell-Cycle Control, and DNA Repair Factors
por: Shkreta, Lulzim, et al.
Publicado: (2016)

MBNL1 reverses the proliferation defect of skeletal muscle satellite cells in myotonic dystrophy type 1 by inhibiting autophagy via the mTOR pathway
por: Song, Kai-Yi, et al.
Publicado: (2020)

High-content screening identifies small molecules that remove nuclear foci, affect MBNL distribution and CELF1 protein levels via a PKC-independent pathway in myotonic dystrophy cell lines
por: Ketley, Ami, et al.
Publicado: (2014)

Daunorubicin reduces MBNL1 sequestration caused by CUG-repeat expansion and rescues cardiac dysfunctions in a Drosophila model of myotonic dystrophy
por: Chakraborty, Mouli, et al.
Publicado: (2018)

Tumor microenvironment–associated modifications of alternative splicing
por: Brosseau, Jean-Philippe, et al.
Publicado: (2014)

Alternative splicing mediates the compensatory upregulation of MBNL2 upon MBNL1 loss-of-function
por: Nitschke, Larissa, et al.
Publicado: (2023)

New function for the RNA helicase p68/DDX5 as a modifier of MBNL1 activity on expanded CUG repeats
por: Laurent, François-Xavier, et al.
Publicado: (2012)

Peptide-conjugated oligonucleotides evoke long-lasting myotonic dystrophy correction in patient-derived cells and mice
por: Klein, Arnaud F., et al.
Publicado: (2019)

Targeting DMPK with Antisense Oligonucleotide Improves Muscle Strength in Myotonic Dystrophy Type 1 Mice
por: Jauvin, Dominic, et al.
Publicado: (2017)

Selective inhibition of MBNL1–CCUG interaction by small molecules toward potential therapeutic agents for myotonic dystrophy type 2 (DM2)(†)
por: Wong, Chun-Ho, et al.
Publicado: (2011)

RNA Splicing Is Responsive to MBNL1 Dose
por: Jog, Sonali P., et al.
Publicado: (2012)

LncRNA MBNL1-AS1 Represses Proliferation and Cancer Stem-Like Properties of Breast Cancer through MBNL1-AS1/ZFP36/CENPA Axis
por: Ding, Yu, et al.
Publicado: (2022)

(CTG)n repeat-mediated dysregulation of MBNL1 and MBNL2 expression during myogenesis in DM1 occurs already at the myoblast stage
por: André, Laurène M., et al.
Publicado: (2019)

Cannot write session to /tmp/vufind_sessions/sess_cbggfrh9ivsj3be8k595jf52e0