Fig. 5
From: A novel homozygous MPV17 mutation in two families with axonal sensorimotor polyneuropathy
Effect of knockdown of MPV17 and overexpression of mutant proteins on cell proliferation. a Confirmation of MPV17 knockdown in NSC34 by RT-PCR. b Inhibitory effect of the proliferation of NSC34 by the abrogation of MPV17 using specific siRNA. c Affection of MPV17 mutations on cell viability against reactive oxygen species (ROS). Knockdown of MPV17 using specific siRNA renders NSC34, a mouse motor neuronal cell line, more sensitive to H2O2 treatment (24 h). d Western blot analysis to determine the expression of mutant proteins. e Overexpression of mutant proteins affected cell proliferation of NSC34. f Changes in the mitochondrial OXPHOS system. Western blotting using OXPHOS detection cocktail antibody was performed after overexpression of wild-type and mutant MPV17 proteins. For cell proliferation and survival assay, 4–6 wells per each sample were counted. Data are presented as mean ± SEM. Statistical analysis were performed using Student’s t-test. * and #, p < 0.05; **and ##, p < 0.01. Statistical significance was determined either with control myc (* and **) or wild-type MPV17 (# and ##) in (e)