Cargando…
An in silico FSHD muscle fiber for modeling DUX4 dynamics and predicting the impact of therapy
Facioscapulohumeral muscular dystrophy (FSHD) is an incurable myopathy linked to the over-expression of the myotoxic transcription factor DUX4. Targeting DUX4 is the leading therapeutic approach, however, it is only detectable in 0.1–3.8% of FSHD myonuclei. How rare DUX4 drives FSHD and the optimal...
Autores principales: | Cowley, Matthew V, Pruller, Johanna, Ganassi, Massimo, Zammit, Peter S, Banerji, Christopher RS |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
eLife Sciences Publications, Ltd
2023
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10287159/ https://www.ncbi.nlm.nih.gov/pubmed/37184373 http://dx.doi.org/10.7554/eLife.88345 |
Ejemplares similares
-
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) -
Antagonism Between DUX4 and DUX4c Highlights a Pathomechanism Operating Through β-Catenin in Facioscapulohumeral Muscular Dystrophy
por: Ganassi, Massimo, et al.
Publicado: (2022) -
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) -
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)