Figure 5.

Increased translation of mutant ATXN3 in SCA3 fibroblasts. (A) Western blot analysis of extracts from fibroblasts derived from a SCA3 patient carrying 74/22 CAG repeats in the ATXN3 gene is shown. Two ATXN3 bands were detected, one smaller translated from the normal, one bigger translated from the mutant allele. Actin was analyzed as control. (B) Quantifications of bands (protein expressed from the normal compared with protein expressed from the mutant allele) from (A) are shown. Mean values ± SEM are shown, n = 3, p < 0.0001. ^*Indicates statistically significant differences (p < 0.05).