Cargando…

A patient-derived iPSC model revealed oxidative stress increases facioscapulohumeral muscular dystrophy-causative DUX4

Double homeobox 4 (DUX4), the causative gene of facioscapulohumeral muscular dystrophy (FSHD), is ectopically expressed in the skeletal muscle cells of FSHD patients because of chromatin relaxation at 4q35. The diminished heterochromatic state at 4q35 is caused by either large genome contractions [F...

Descripción completa

Detalles Bibliográficos
Autores principales:	Sasaki-Honda, Mitsuru, Jonouchi, Tatsuya, Arai, Meni, Hotta, Akitsu, Mitsuhashi, Satomi, Nishino, Ichizo, Matsuda, Ryoichi, Sakurai, Hidetoshi
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Oxford University Press 2018
Materias:	General Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6240734/ https://www.ncbi.nlm.nih.gov/pubmed/30107443 http://dx.doi.org/10.1093/hmg/ddy293

Ejemplares similares

Establishment of quantitative and consistent in vitro skeletal muscle pathological models of myotonic dystrophy type 1 using patient-derived iPSCs
por: Kawada, Ryu, et al.
Publicado: (2023)

DUX4 Signalling in the Pathogenesis of Facioscapulohumeral Muscular Dystrophy
por: Lim, Kenji Rowel Q., et al.
Publicado: (2020)

Nanopore direct RNA sequencing detects DUX4-activated repeats and isoforms in human muscle cells
por: Mitsuhashi, Satomi, et al.
Publicado: (2021)

Influence of DUX4 Expression in Facioscapulohumeral Muscular Dystrophy and Possible Treatments
por: Duranti, Elisa, et al.
Publicado: (2023)

Characterization of hiPSC-Derived Muscle Progenitors Reveals Distinctive Markers for Myogenic Cell Purification Toward Cell Therapy
por: Nalbandian, Minas, et al.
Publicado: (2021)

Morpholino-mediated Knockdown of DUX4 Toward Facioscapulohumeral Muscular Dystrophy Therapeutics
por: Chen, Jennifer CJ, et al.
Publicado: (2016)

Pathomechanisms and biomarkers in facioscapulohumeral muscular dystrophy: roles of DUX4 and PAX7
por: Banerji, Christopher R S, et al.
Publicado: (2021)

Identification of Epigenetic Regulators of DUX4-fl for Targeted Therapy of Facioscapulohumeral Muscular Dystrophy
por: Himeda, Charis L., et al.
Publicado: (2018)

A Deoxyribonucleic Acid Decoy Trapping DUX4 for the Treatment of Facioscapulohumeral Muscular Dystrophy
por: Mariot, Virginie, et al.
Publicado: (2020)

CRISPR mediated targeting of DUX4 distal regulatory element represses DUX4 target genes dysregulated in Facioscapulohumeral muscular dystrophy
por: Das, Sunny, et al.
Publicado: (2021)

Antagonism Between DUX4 and DUX4c Highlights a Pathomechanism Operating Through β-Catenin in Facioscapulohumeral Muscular Dystrophy
por: Ganassi, Massimo, et al.
Publicado: (2022)

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

p53-independent DUX4 pathology in cell and animal models of facioscapulohumeral muscular dystrophy
por: Bosnakovski, Darko, et al.
Publicado: (2017)

A cre-inducible DUX4 transgenic mouse model for investigating facioscapulohumeral muscular dystrophy
por: Jones, Takako, et al.
Publicado: (2018)

Systemic delivery of a DUX4-targeting antisense oligonucleotide to treat facioscapulohumeral muscular dystrophy
por: Bouwman, Linde F., et al.
Publicado: (2021)

RIPK3‐mediated cell death is involved in DUX4‐mediated toxicity in facioscapulohumeral dystrophy
por: Mariot, Virginie, et al.
Publicado: (2021)

A muscle fatigue-like contractile decline was recapitulated using skeletal myotubes from Duchenne muscular dystrophy patient-derived iPSCs
por: Uchimura, Tomoya, et al.
Publicado: (2021)

A feedback loop between nonsense-mediated decay and the retrogene DUX4 in facioscapulohumeral muscular dystrophy
por: Feng, Qing, et al.
Publicado: (2015)

DUX4 promotes transcription of FRG2 by directly activating its promoter in facioscapulohumeral muscular dystrophy
por: Thijssen, Peter E, et al.
Publicado: (2014)

Adenine base editing of the DUX4 polyadenylation signal for targeted genetic therapy in facioscapulohumeral muscular dystrophy
por: Šikrová, Darina, et al.
Publicado: (2021)

Dual CRISPR-Cas3 system for inducing multi-exon skipping in DMD patient-derived iPSCs
por: Kita, Yuto, et al.
Publicado: (2023)

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

Identification of candidate miRNA biomarkers for facioscapulohumeral muscular dystrophy using DUX4-based mouse models
por: Nunes, Andreia M., et al.
Publicado: (2021)

Analysis of genes regulated by DUX4 via oxidative stress reveals potential therapeutic targets for treatment of facioscapulohumeral dystrophy
por: Karpukhina, Anna, et al.
Publicado: (2021)

Nanopore-based single molecule sequencing of the D4Z4 array responsible for facioscapulohumeral muscular dystrophy
por: Mitsuhashi, Satomi, et al.
Publicado: (2017)

Transgenic mice expressing tunable levels of DUX4 develop characteristic facioscapulohumeral muscular dystrophy-like pathophysiology ranging in severity
por: Jones, Takako I., et al.
Publicado: (2020)

A case of type 1 facioscapulohumeral muscular dystrophy (FSHD) with restrictive ventilatory defect and congestive heart failure
por: Morimoto, Nobutoshi, et al.
Publicado: (2020)

Transplantation of human iPSC-derived muscle stem cells in the diaphragm of Duchenne muscular dystrophy model mice
por: Miura, Yasutomo, et al.
Publicado: (2022)

Multiple Protein Domains Contribute to Nuclear Import and Cell Toxicity of DUX4, a Candidate Pathogenic Protein for Facioscapulohumeral Muscular Dystrophy
por: Corona, Edgardo Daniel, et al.
Publicado: (2013)

DUX4-induced dsRNA and MYC mRNA stabilization activate apoptotic pathways in human cell models of facioscapulohumeral dystrophy
por: Shadle, Sean C., et al.
Publicado: (2017)

A Targeted Approach for Evaluating DUX4-Regulated Proteins as Potential Serum Biomarkers for Facioscapulohumeral Muscular Dystrophy Using Immunoassay Proteomics
por: Campbell, Amy E., et al.
Publicado: (2023)

Causative variant profile of collagen VI-related dystrophy in Japan
por: Inoue, Michio, et al.
Publicado: (2021)

Human miRNA miR-675 inhibits DUX4 expression and may be exploited as a potential treatment for Facioscapulohumeral muscular dystrophy
por: Saad, Nizar Y., et al.
Publicado: (2021)

Ophthalmological findings in facioscapulohumeral dystrophy
por: Goselink, Rianne J M, et al.
Publicado: (2019)

Human iPSC Models to Study Orphan Diseases: Muscular Dystrophies
por: Xia, Guangbin, et al.
Publicado: (2018)

Treatment of facioscapulohumeral muscular dystrophy with Denosumab
por: Lefkowitz, Stanley S., et al.
Publicado: (2012)

Facioscapulohumeral dystrophy: the path to consensus on pathophysiology
por: Tawil, Rabi, et al.
Publicado: (2014)

Sarcopenic Obesity in Facioscapulohumeral Muscular Dystrophy
por: Vera, Kathryn, et al.
Publicado: (2020)

Characterizing the face in facioscapulohumeral muscular dystrophy
por: Loonen, T. G. J., et al.
Publicado: (2020)

The socioeconomic burden of facioscapulohumeral muscular dystrophy
por: Blokhuis, Anna M., et al.
Publicado: (2021)

Cannot write session to /tmp/vufind_sessions/sess_imbd3holjklnt0h5fom51et73i