Effects of wild-type MECP2 or MBD-deleted mutations on the transcriptional activity of the methylated or unmethylated PCDHB1 and PCDH7 promoter fragments. SH-SY5Y cells were transfected with the methylated or unmethylated PCDHB1-luc (A), PCDH7-luc (B) as a reporter vector. As an effector, an MECP2-expression vector was co-transfected. After 48 h, the transfectants were lysed and assayed for luciferase activity. PCDHB1 and PCDH7 promoter transcriptional activities are repressed by wild-type MeCP2 (gray), but not by MBD-deleted mutant (black) in SH-SY5Y cells. Luciferase reporter activity in each sample was normalized according to the beta-galactosidase activity measured in the same sample. The luciferase activity of the cells transfected with the reporter vector was taken as 100%. All results are shown as the mean ± SEM of three replicates.