Fig. 3

DSP mRNA expression, total DSP and JUP protein expression. HEK293T cells were transfected with either blank, wild type or mutant plasmids. Blank plasmids without DSP gene served as control group. a-b qPCR analysis for DSP mRNA levels in the N-terminal and C-terminal of the c.832delG mutation site. There were no significant differences between mutant and wild-type in mRNA levels in the N-terminal side of DSP mutation, whereas, mRNA level in C-terminal side of DSP mutation was only elevated in cells transfected with wild type plasmid transfection; c-dGFP antibody was used to exam the length of protein expressed in whole cell lysates. Mutant DSP protein was much shorter than wild type, suggesting truncation effect of the mutation. JUP expression was significantly increased in the mutant group. GAPDH served as an internal control. DSP, Desmoplakin; JUP, Junction plakoglobin; WT, wild type