Journal article

Misfolded alpha-synuclein causes hyperactive respiration without functional deficit in live neuroblastoma cells

Cathryn L Ugalde, Sarah J Annesley, Shane E Gordon, Katelyn Mroczek, Matthew A Perugini, Victoria A Lawson, Paul R Fisher, David I Finkelstein, Andrew F Hill

Disease Models and Mechanisms | COMPANY BIOLOGISTS LTD | Published : 2020


The misfolding and aggregation of the largely disordered protein, α-synuclein, is a central pathogenic event that occurs in the synucleinopathies, a group of neurodegenerative disorders that includes Parkinson's disease. While there is a clear link between protein misfolding and neuronal vulnerability, the precise pathogenic mechanisms employed by disease-associated α-synuclein are unresolved. Here, we studied the pathogenicity of misfolded α-synuclein produced using the protein misfolding cyclic amplification (PMCA) assay. To do this, previous published methods were adapted to allow PMCA-induced protein fibrillization to occur under non-toxic conditions. Insight into potential intracellular..

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