Overexpression of ubiquilin-2 proteins carrying ALS mutations increase cell death.

<p>HeLa cell cultures plated at similar density were either transfected with an empty vector (control), or with cDNAs encoding WT ubiquilin-2 or each of the five different ALS mutations. 24 after transfection the cells were stained with Hoechst and the expressed ubiquilin-2 proteins to identify dying cells and the transfection efficiency, respectively. The transfection efficiency in these experiments was >95% (not shown). Representative examples showing an increase in the proportion of cells that had bright abnormal nuclear morphology by Hoechst staining. Bar = 50 μm. (B) Graph represents the total number of cells that had abnormal bright nuclear morphology (excluding mitotic cells) as a percentage of the total number of cells. Errors bars show standard error of the mean. The * signifies a statistical increase in cell death compared with the mock transfected cells (p = <0.05).