Fig. 6.

Overexpression of Hsp40 chaperones reduces aggregation of truncated and full-length ataxin-3. COS7 cells were cotransfected with plasmids encoding HA-Q78 (A,C, E) or NLS-ataxin-3 (Q78) (B, D, F), together with empty control plasmid or plasmids encoding HDJ-1 (A, B), HDJ-2 (C,D), or Hsp70 (E,F). Suppression of aggregate formation occurred with HDJ-1 and HDJ-2 but not Hsp70. A partially deleted form of HDJ-2 lacking the DnaJ domain (del9–107) also suppressed aggregation of mutant ataxin-3. Shown are the mean values of three independent experiments. Statistically significant differences, determined by paired t test, are indicated by asterisks(*p < 0.05; **p < 0.01).