Table 1. Detergent-resistant SOD1 aggregates in fibroblast cultures.
| Proteasome inhibition | Autophagy inhibition | ER-stress induction | Inhibition of mitochondria | |||||
|---|---|---|---|---|---|---|---|---|
| Bortezomib | 3-MA | Tunicamycin | Rotenone | |||||
| - | + | - | + | - | + | - | + | |
| A4V | <0.03 | 0.39 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 |
| H46R | <0.03 | 0.09 | 0.15 | 0.14 | <0.03 | <0.03 | 0.09 | 0.23 |
| N86S | <0.03 | <0.03 | n. t. | n. t. | <0.03 | <0.03 | n. t. | n. t. |
| D90A | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 |
| G93A | <0.03 | 0.04 | <0.03 | <0.03 | n. t. | n. t. | n. t. | n. t. |
| L117V | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 |
| G127X | 0.20 | 0.75 | 0.08 | 0.06 | <0.03 | <0.03 | <0.03 | <0.03 |
| E78_R79insSI | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 |
| D125Tfs*24 | 0.22 | 25.26 | 0.49 | 0.24 | 0.73 | 0.41 | 0.35 | 0.30 |
| C9orf72exp | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 |
| C9orf72exp | <0.03 | <0.03 | n. t. | n. t. | <0.03 | <0.03 | n. t. | n. t. |
| Control | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 | <0.03 |
The detergent-resistant aggregates were quantified from western blots. Amounts are presented as % of total amounts of SOD1 in the cultures as measured by ELISA. The detection limit for SOD1 aggregates in the cultures was approximately 0.03% of total SOD1, n. t. = not tested.