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C9ORF72-derived poly-GA DPRs undergo endocytic uptake in iAstrocytes and spread to motor neurons
Dipeptide repeat (DPR) proteins are aggregation-prone polypeptides encoded by the pathogenic GGGGCC repeat expansion in the C9ORF72 gene, the most common genetic cause of amyotrophic lateral sclerosis and frontotemporal dementia. In this study, we focus on the role of poly-GA DPRs in disease spread....
Autores principales: | Marchi, Paolo M, Marrone, Lara, Brasseur, Laurent, Coens, Audrey, Webster, Christopher P, Bousset, Luc, Destro, Marco, Smith, Emma F, Walther, Christa G, Alfred, Victor, Marroccella, Raffaele, Graves, Emily J, Robinson, Darren, Shaw, Allan C, Wan, Lai Mei, Grierson, Andrew J, Ebbens, Stephen J, De Vos, Kurt J, Hautbergue, Guillaume M, Ferraiuolo, Laura, Melki, Ronald, Azzouz, Mimoun |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Life Science Alliance LLC
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9108631/ https://www.ncbi.nlm.nih.gov/pubmed/35568435 http://dx.doi.org/10.26508/lsa.202101276 |
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