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Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUS(WT) transgenic mice

FUS (fused in sarcoma) mislocalization and cytoplasmic aggregation are hallmark pathologies in FUS-related amyotrophic lateral sclerosis and frontotemporal dementia. Many of the mechanistic hypotheses have focused on a loss of nuclear function in the FUS-opathies, implicating dysregulated RNA transc...

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Detalles Bibliográficos
Autores principales:	So, Eva, Mitchell, Jacqueline C, Memmi, Caroline, Chennell, George, Vizcay-Barrena, Gema, Allison, Leanne, Shaw, Christopher E, Vance, Caroline
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Oxford University Press 2018
Materias:	Articles
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5886082/ https://www.ncbi.nlm.nih.gov/pubmed/29194538 http://dx.doi.org/10.1093/hmg/ddx415

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