Fig. 1. Proposed model of self-propagation of misfolded protein fibrils in ALS. Misfolded protein aggregates bind to their native counterparts at their end and induce the misfolding of captured protein in a template-directed reaction. This process elongates the misfolded protein fibrils. Amplification of self-templating amyloid-like fibrils is achieved by the fragmentation of amyloid forms to expose new ends. Breakage of misfolded fibrils also allows the dissemination of self-propagating seeds.
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