Fig. 6

Mutant huntingtin inhibits the binding of Sp1 to ZnT3 promoter. a Dual-luciferase report gene assay shows that the promoter activity of ZnT3 is down-regulated in BHK cells transfected with different concentrations of 160Q Htt vector. n = 3. *p < 0.05 compared to 20Q cells. b Dual-luciferase report gene assay shows that Sp1 overexpression up-regulates ZnT3 promoter transcriptional activity in 160Q BHK cells. n = 3. c ChIP analysis shows that mHtt inhibits the binding of Sp1 to ZnT3 promoter containing GC-1 box or GC-2 box. The relative amounts of ZnT3 were normalized to input DNA. n = 3. *p < 0.05 compared to 20Q Htt. d RT-PCR analysis shows that forced overexpressed Sp1 reverses the suppression of ZnT3 mRNA by mHtt in 160Q BHK cells. n = 3, *p < 0.05 compared to 20Q cells. #p < 0.05 compared to 160Q cells