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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....

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Detalles Bibliográficos
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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Life Science Alliance LLC 2022
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