Transfection of mutant FUS is associated with SOD1 misfolding by immunocytochemistry.

<p>Human neuroblastoma SH-SY5Y cells and primary neural cultures expressing human wtSOD1 were stained for HA-Tag (red), misfolded SOD1 (green), and Hoechst33342 nuclear counterstain (blue). (<b>A</b>, <b>B</b>) Human wild-type FUS localizes in the nucleus and no misfolded SOD1 is detected. (<b>C</b>–<b>F</b>) Both of the truncated variant, R495x-FUS (<b>B</b>, <b>C</b>), and point mutation variant, P525L-FUS (<b>E</b>, <b>F</b>), localize in the cytosol and are associated with misfolding of SOD1 in the same cells, as detected by the immunocytochemistry with the 3H1 SOD1 misfolding-specific mAb. Exogenous FUS was detected using the N-terminal HA-tag. Arrows point to transfected cells. Scale bar, 20µm.