Fig. 9

Toxicity of CSF samples from sALS toward motor neuronal cell line comes from misfolded SOD1. (a) Differentiated NSC-34 cells were exposed to (white bars) the CSF samples, (gray bars) the CSF samples preabsorbed with normal mouse IgG, and (black bars) the CSF samples preabsorbed with mouse monoclonal C4F6 antibody for 48 h, and the cell viability was assayed with Cell Counting Kit-8. Each of the CSF samples was tested in duplicate, and the data are shown as the averaged cell viability relative to that of the negative control, in which PBS instead of CSF samples was exposed to the cells. (b-g) Representative images of the differentiated NSC-34 cells exposed to the CSF samples from (b-d) non-ND (C-15) and (e-g) sALS (ALS13) are shown. The cells were incubated with (b, e) the CSF samples, (c, f) the CSF samples preabsorbed with mouse IgG, and (d, g) the CSF samples preabsorbed with mouse monoclonal C4F6 antibody. The dying cells were stained with DAPI (shown in blue)