Cargando…
Neuronal over-expression of Oxr1 is protective against ALS-associated mutant TDP-43 mislocalisation in motor neurons and neuromuscular defects in vivo
A common pathological hallmark of amyotrophic lateral sclerosis (ALS) and the related neurodegenerative disorder frontotemporal dementia, is the cellular mislocalization of transactive response DNA-binding protein 43 kDa (TDP-43). Additionally, multiple mutations in the TARDBP gene (encoding TDP-43)...
Autores principales: | Williamson, Matthew G, Finelli, Mattéa J, Sleigh, James N, Reddington, Amy, Gordon, David, Talbot, Kevin, Davies, Kay E, Oliver, Peter L |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2019
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6927465/ https://www.ncbi.nlm.nih.gov/pubmed/31642482 http://dx.doi.org/10.1093/hmg/ddz190 |
Ejemplares similares
-
Oxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutations
por: Finelli, Mattéa J., et al.
Publicado: (2015) -
p62 overexpression induces TDP-43 cytoplasmic mislocalisation, aggregation and cleavage and neuronal death
por: Foster, A. D., et al.
Publicado: (2021) -
Overexpression of survival motor neuron improves neuromuscular function and motor neuron survival in mutant SOD1 mice
por: Turner, Bradley J., et al.
Publicado: (2014) -
Neuron-specific antioxidant OXR1 extends survival of a mouse model of amyotrophic lateral sclerosis
por: Liu, Kevin X., et al.
Publicado: (2015) -
Oxr1 Is Essential for Protection against Oxidative Stress-Induced Neurodegeneration
por: Oliver, Peter L., et al.
Publicado: (2011)