Article
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.
Acta neuropathologica communications - 20 May 2021
Ayers Jacob I, Xu Guilian, Dillon Kristy, Lu Qing, Chen Zhijuan, Beckman John, Moreno-Romero Alma K, Zamora Diana L, Galaleldeen Ahmad, Borchelt David R
Abstract excerpt
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 that the efficiency of transmission is governed by the primary sequence of prion protein (PrP). Isologous seeding, where the...
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