C9orf72 poly(PR) mediated neurodegeneration is associated with nucleolar stress

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The ALS/FTD-linked intronic hexanucleotide repeat expansion in the C9orf72 gene is translated into dipeptide repeat proteins, among which poly-proline-arginine (PR) displays the most aggressive neurotoxicity in-vitro and in-vivo. PR partitions to the nucleus when expressed in neurons and other cell...

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Detalles Bibliográficos
Autores principales: Cicardi, ME, Hallgren, JH, Mawrie, D, Krishnamurthy, K, Markandaiah, SS, Nelson, AT, Kankate, V, Anderson, EN, Pasinelli, P, Pandey, UB, Eischen, CM, Trotti, D
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cold Spring Harbor Laboratory 2023
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Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9949130/
https://www.ncbi.nlm.nih.gov/pubmed/36824930
http://dx.doi.org/10.1101/2023.02.16.528809
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