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Article

A mouse model with widespread expression of the C9orf72-linked glycine-arginine dipeptide displays non-lethal ALS/FTD-like phenotypes.

Scientific reports - 4 Apr 2022

Verdone Brandie Morris, Cicardi Maria Elena, Wen Xinmei, Sriramoji Sindhu, Russell Katelyn, Markandaiah Shashirekha S, Jensen Brigid K, Krishnamurthy Karthik, Haeusler Aaron R, Pasinelli Piera, Trotti Davide

Abstract excerpt

Translation of the hexanucleotide G4C2 expansion associated with C9orf72 amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD) produces five different dipeptide repeat protein (DPR) species that can confer toxicity. There is yet much to learn about the contribution of a single DPR to disease pathogenesis. We show here that a short repeat length is sufficient for the DPR poly-GR to confer...

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PMID: 35379876
DOI: 10.1038/s41598-022-09593-z
PMCID: PMC8979946

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