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

Summary: The ALS/FTD-linked intronic hexanucleotide repeat expansion in the C9orf72 gene is aberrantly translated in the sense and antisense directions into dipeptide repeat proteins, among which poly proline-arginine (PR) displays the most aggressive neurotoxicity in-vitro and in-vivo. PR partition...

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Bibliographic Details
Main Authors: M.E. Cicardi, J.H. Hallgren, D. Mawrie, K. Krishnamurthy, S.S. Markandaiah, A.T. Nelson, V. Kankate, E.N. Anderson, P. Pasinelli, U.B. Pandey, C.M. Eischen, D. Trotti
Format: Article
Language:English
Published: Elsevier 2023-09-01
Series:iScience
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2589004223015821