Fig. 6.

Sensitivity to protein misfolding. The fibroblasts were exposed to canavanine and followed by continuous cell monitoring. Representative images are shown in (A) and quantifications in (B). After 24 h of canavanine treatment, HSPB1^∆C-term fibroblasts started to die (rounded cells, arrowheads) and after 48 h the same was seen for control and HSPB1^R127L fibroblasts. Quantification showed that the number of cells with normal flat morphology decreased in all cell lines but significantly more in HSPB1^∆C-term fibroblasts. Conversely, the number of dying (round) cells increased significantly more in HSPB1^∆C-term fibroblasts compared to the other cell lines. Asterisks denotes significant difference compared to control fibroblasts *P < 0.01, **P < 0.0001 (two-way ANOVA).