Is Multiple System Atrophy a Prion Disorder?

Prions are self-propagating conformational strains of proteins that can transmit themselves into other organisms, propagate through multiple generations, and produce the same phenotype. Prions are considered the causative agent in Creutzfeldt-Jakob disease (CJD). Prion-like propagation has been suggested as the mechanism of spread of other neurodegenerative disorders, such as alpha-synucleinopathies and tauopathies (Proc Natl Acad Sci U S A 2013; 110:19555, and Neuron 2014; 82:1271). In this study, researchers sought to determine whether inoculating transgenic mice (expressing mutant human alpha synuclein A53T protein) and human embryonic kidney (HEK) cells (expressing alpha-synuclein fused to yellow fluorescent protein) with homogenates fr…