Extended Data Fig. 9: Altered p300 localisation, AMPK activity and CRM1 expression in HGPS cells.
a, Decreased Lamin B1 expression level in HGPS cells. * GFP-progerin induced cells. Images are representative of three biologically independent experiments (N = 3). Scale bar, 5 μm. b, c, Defect in nucleus-cytoplasmic transport by progerin using RAN staining (b) or shuttling-reporter construct (NLS-tdTomato-NES) (c). N = 3. Scale bar, 5 μm. d, Altered p300 localisation by progerin expression using fractionation. N = 3. Two-tailed paired t test. e, Differential regulation of acetylated proteins between nucleus and cytoplasm in progerin-expressing cells. N = 3. One sample t-test. f, Enhanced CRM1 expression in HGPS cells. N = 3, about 50 cells scored per condition. One sample t-test. Scale bar, 5 μm. g, Defect in AMPK activation and increased CRM1 expression in HGPS fibroblasts. N = 3. One-way ANOVA with post hoc Tukey test. Data from control vials #1 and #2 were pooled as these are from the same individual (see Supplementary Table 1). h, Rescue of enhanced p300 activity in cytoplasm after treatment with A485, 991 or SEL. N = 4 each group. One-way ANOVA with post hoc Tukey test. i, Reduced progerin expression levels in HGPS fibroblasts by treatment with p300 inhibitor A485 for 24 h. N = 3. Two-tailed paired t test. j, Reduced Lamin B1 levels in HGPS fibroblasts. Scale bar, 5 μm, 2 μm (enlarged images). N = 3, about 50 cells scored per condition. Data are presented as mean values +/- s.d. unless otherwise specified. Source numerical data and unprocessed blots are available in source data.
