Variation in the vulnerability of mice expressing human superoxide dismutase 1 to prion-like seeding: a study of the influence of primary amino acid sequence

Abstract Misfolded forms of superoxide dismutase 1 (SOD1) with mutations associated with familial amyotrophic lateral sclerosis (fALS) exhibit prion characteristics, including the ability to act as seeds to accelerate motor neuron disease in mouse models. A key feature of infectious prion seeding is...

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Bibliographic Details
Main Authors: Jacob I. Ayers, Guilian Xu, Kristy Dillon, Qing Lu, Zhijuan Chen, John Beckman, Alma K. Moreno-Romero, Diana L. Zamora, Ahmad Galaleldeen, David R. Borchelt
Format: Article
Language:English
Published: BMC 2021-05-01
Series:Acta Neuropathologica Communications
Online Access:https://doi.org/10.1186/s40478-021-01191-w