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Article

Prion protein pathology in Ubiquilin 2 models of ALS.

Neurobiology of disease - 15 Oct 2024

Le Nhat T, Chu Nam, Joshi Gunjan, Higgins Nicole R, Nebie Ouada, Adelakun Niyi, Butts Marie, Monteiro Mervyn J

Abstract excerpt

Mutations in UBQLN2 cause ALS and frontotemporal dementia (FTD). The pathological signature in UBQLN2 cases is deposition of highly unusual types of inclusions in the brain and spinal cord that stain positive for UBQLN2. However, what role these inclusions play in pathogenesis remains unclear. Here we show cellular prion protein (PrPC) is found in UBQLN2 inclusions in both mouse and human neuronal induced...

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Identifiers

PMID: 39299489
DOI: 10.1016/j.nbd.2024.106674
PMCID: PMC11651290

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