CNS-derived extracellular vesicles from superoxide dismutase 1 (SOD1)G93A ALS mice originate from astrocytes and neurons and carry misfolded SOD1

Authors: Judith M. Silverman, Darren Christy, Chih Cheih Shyu, Kyung‐Mee Moon, Sarah Fernando, Zoe Gidden, Catherine M. Cowan, Yuxin Ban, R. Greg Stacey, Leslie I. Grad, Luke McAlary, Ian R. Mackenzie, Leonard J. Foster, Neil R. Cashman - Journal of Biological Chemistry 2019 cited by 158

ALS mouse model, as well as from human SOD1 familial ALS patient spinal cord, contained abundant misfolded and nonnative disulfide-cross-linked aggregated SOD1. Our results indicate that CNS-derived EVs from an ALS animal model contain pathogenic disease-causing proteins and suggest that brain astrocytes and neurons, but not microglia, are the main EV source.