Fig 4. Inhibition of proteasome causes accumulation of detergent-insoluble SOD1 aggregates.

Fibroblast cell lines were cultured in the absence (-) and presence (+) of bortezomib (5 ng/ml) for 24 h. Detergent insoluble fractions were analysed by western blotting using the anti-SOD1 (57–72 aa) antibody. No insoluble aggregates were seen in the control and D90A lines. Proteasome inhibition increased the amount of aggregates in G93A, A4V, H46R, G127X and D125Tfs*24 lines.