Cargando…

DUX4 expression in FSHD muscle cells: how could such a rare protein cause a myopathy?

Facioscapulohumeral muscular dystrophy (FSHD) is one of the most frequent hereditary muscle disorders. It is linked to contractions of the D4Z4 repeat array in 4q35. We have characterized the double homeobox 4 (DUX4) gene in D4Z4 and its mRNA transcribed from the distal D4Z4 unit to a polyadenylatio...

Descripción completa

Detalles Bibliográficos
Autores principales:	Tassin, Alexandra, Laoudj-Chenivesse, Dalila, Vanderplanck, Céline, Barro, Marietta, Charron, Sébastien, Ansseau, Eugénie, Chen, Yi-Wen, Mercier, Jacques, Coppée, Frédérique, Belayew, Alexandra
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Blackwell Publishing Ltd 2013
Materias:	Original Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3823138/ https://www.ncbi.nlm.nih.gov/pubmed/23206257 http://dx.doi.org/10.1111/j.1582-4934.2012.01647.x

Ejemplares similares

The FSHD Atrophic Myotube Phenotype Is Caused by DUX4 Expression
por: Vanderplanck, Céline, et al.
Publicado: (2011)

DUX4c Is Up-Regulated in FSHD. It Induces the MYF5 Protein and Human Myoblast Proliferation
por: Ansseau, Eugénie, et al.
Publicado: (2009)

Overexpression of the double homeodomain protein DUX4c interferes with myofibrillogenesis and induces clustering of myonuclei
por: Vanderplanck, Céline, et al.
Publicado: (2018)

Antisense Oligonucleotides Used to Target the DUX4 mRNA as Therapeutic Approaches in FaciosScapuloHumeral Muscular Dystrophy (FSHD)
por: Ansseau, Eugénie, et al.
Publicado: (2017)

FSHD Myotubes with Different Phenotypes Exhibit Distinct Proteomes
por: Tassin, Alexandra, et al.
Publicado: (2012)

Homologous Transcription Factors DUX4 and DUX4c Associate with Cytoplasmic Proteins during Muscle Differentiation
por: Ansseau, Eugénie, et al.
Publicado: (2016)

The double homeodomain protein DUX4c is associated with regenerating muscle fibers and RNA-binding proteins
por: Claus, Clothilde, et al.
Publicado: (2023)

Myoblasts from affected and non-affected FSHD muscles exhibit morphological differentiation defects
por: Barro, Marietta, et al.
Publicado: (2010)

Dux4 controls migration of mesenchymal stem cells through the Cxcr4-Sdf1 axis
por: Dmitriev, Petr, et al.
Publicado: (2016)

Induction of a local muscular dystrophy using electroporation in vivo: an easy tool for screening therapeutics
por: Derenne, Aline, et al.
Publicado: (2020)

Therapeutic Strategies Targeting DUX4 in FSHD
por: Le Gall, Laura, et al.
Publicado: (2020)

Functional domains of the FSHD-associated DUX4 protein
por: Mitsuhashi, Hiroaki, et al.
Publicado: (2018)

Culture Conditions Affect Expression of DUX4 in FSHD Myoblasts
por: Pandey, Sachchida Nand, et al.
Publicado: (2015)

DUX4 Role in Normal Physiology and in FSHD Muscular Dystrophy
por: Mocciaro, Emanuele, et al.
Publicado: (2021)

Designed U7 snRNAs inhibit DUX4 expression and improve FSHD-associated outcomes in DUX4 overexpressing cells and FSHD patient myotubes
por: Rashnonejad, Afrooz, et al.
Publicado: (2020)

Systemic antisense therapeutics inhibiting DUX4 expression ameliorates FSHD-like pathology in an FSHD mouse model
por: Lu-Nguyen, Ngoc, et al.
Publicado: (2021)

MATR3 is an endogenous inhibitor of DUX4 in FSHD muscular dystrophy
por: Runfola, Valeria, et al.
Publicado: (2023)

Aberrant Splicing in Transgenes Containing Introns, Exons, and V5 Epitopes: Lessons from Developing an FSHD Mouse Model Expressing a D4Z4 Repeat with Flanking Genomic Sequences
por: Ansseau, Eugénie, et al.
Publicado: (2015)

DUX4 Binding to Retroelements Creates Promoters That Are Active in FSHD Muscle and Testis
por: Young, Janet M., et al.
Publicado: (2013)

DUX4 Expression in FSHD Muscles: Focus on Its mRNA Regulation
por: Sidlauskaite, Eva, et al.
Publicado: (2020)

Gene Editing Targeting the DUX4 Polyadenylation Signal: A Therapy for FSHD?
por: Joubert, Romains, et al.
Publicado: (2020)

DUX4 expressing immortalized FSHD lymphoblastoid cells express genes elevated in FSHD muscle biopsies, correlating with the early stages of inflammation
por: Banerji, Christopher R S, et al.
Publicado: (2020)

Biphasic Myopathic Phenotype of Mouse DUX, an ORF within Conserved FSHD-Related Repeats
por: Bosnakovski, Darko, et al.
Publicado: (2009)

PARP1 Differentially Interacts with Promoter region of DUX4 Gene in FSHD Myoblasts
por: Sharma, Vishakha, et al.
Publicado: (2016)

DNA aptamers against the DUX4 protein reveal novel therapeutic implications for FSHD
por: Klingler, Christian, et al.
Publicado: (2020)

An in silico FSHD muscle fiber for modeling DUX4 dynamics and predicting the impact of therapy
por: Cowley, Matthew V, et al.
Publicado: (2023)

Telomere Position Effect (TPE) Regulates DUX4 in Human Facioscapulohumeral Muscular Dystrophy (FSHD)
por: Stadler, Guido, et al.
Publicado: (2013)

Expression of FSHD-related DUX4-FL alters proteostasis and induces TDP-43 aggregation
por: Homma, Sachiko, et al.
Publicado: (2015)

Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model
por: Bosnakovski, Darko, et al.
Publicado: (2017)

WDR5 is required for DUX4 expression and its pathological effects in FSHD muscular dystrophy
por: Mocciaro, Emanuele, et al.
Publicado: (2023)

DUX4 Differentially Regulates Transcriptomes of Human Rhabdomyosarcoma and Mouse C2C12 Cells
por: Sharma, Vishakha, et al.
Publicado: (2013)

Nuclear bodies reorganize during myogenesis in vitro and are differentially disrupted by expression of FSHD-associated DUX4
por: Homma, Sachiko, et al.
Publicado: (2016)

Proximity ligation assay to detect DUX4 protein in FSHD1 muscle: a pilot study
por: Beermann, Mary Lou, et al.
Publicado: (2022)

Transgenic Drosophila for Investigating DUX4 and FRG1, Two Genes Associated with Facioscapulohumeral Muscular Dystrophy (FSHD)
por: Jones, Takako I., et al.
Publicado: (2016)

Author Correction: Muscle pathology from stochastic low level DUX4 expression in an FSHD mouse model
por: Bosnakovski, Darko, et al.
Publicado: (2018)

PAX7 target gene repression is a superior FSHD biomarker than DUX4 target gene activation, associating with pathological severity and identifying FSHD at the single-cell level
por: Banerji, Christopher R S, et al.
Publicado: (2019)

Asymmetric Bidirectional Transcription from the FSHD-Causing D4Z4 Array Modulates DUX4 Production
por: Block, Gregory J., et al.
Publicado: (2012)

Targeted epigenetic repression by CRISPR/dSaCas9 suppresses pathogenic DUX4-fl expression in FSHD
por: Himeda, Charis L., et al.
Publicado: (2020)

Persistent Fibroadipogenic Progenitor Expansion Following Transient DUX4 Expression Provokes a Profibrotic State in a Mouse Model for FSHD
por: Bosnakovski, Darko, et al.
Publicado: (2022)

A Systemically Administered Unconjugated Antisense Oligonucleotide Targeting DUX4 Improves Muscular Injury and Motor Function in FSHD Model Mice
por: Kakimoto, Tetsuhiro, et al.
Publicado: (2023)

Cannot write session to /tmp/vufind_sessions/sess_228ukne7k0ca08t9f1ibe7pn6p