Decreased steady-state levels of <i>Rgs8</i> message and protein in BAC-Q72 mice.

<p><b>(A)</b> qRT-PCR analyses of cerebellar RNAs from wild-type and BAC-Q22 mice show unchanged <i>Rgs8</i> levels, whereas BAC-Q72 mice show significant and progressive reduction of <i>Rgs8</i> mRNA levels starting at 5 weeks of age. n: number of animals in each group. The data are means ± SD, **p<0.01, ***p<0.001. <b>(B)</b> Western blot analyses indicate reduction of Rgs8 steady-state levels in cerebella of BAC-Q72 mice, but no change in BAC-Q22 mice when compared with wild-type mice. The blot is a representative Western blot of 3 independently performed experiments with 2 animals each per BAC line. <b>(C)</b> SCA2 patient-derived LB cells demonstrate decreased <i>RGS8</i> transcripts. Total RNAs were isolated from LB cell lines derived from two normal control individuals and two SCA2 patients and subjected to RT-PCR analysis using primers specifically amplifying the human <i>ATXN2</i> CAG repeat. RT-PCR analyses indicate the expression of ATXN2 with expanded CAG repeats (46 or 52) (left panel). qRT-PCR analyses of synthesized cDNAs from LB cells show significant reduction of <i>RGS8</i> in both SCA2-LB cell lines. The data represent mean ± SD, **p<0.01 (right panel).