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Defective Lamin A-Rb Signaling in Hutchinson-Gilford Progeria Syndrome and Reversal by Farnesyltransferase Inhibition

Figure 6

Detection of lamin A/C and Rb in cells from subjects with HGPS and in normal human skin biopsies.

(A) Left panel: Western blot of proteins extracted from control fibroblasts (Control-1 [GM02036A], Control-2 [GM03349C]) and fibroblasts from subjects with HGPS (HGADFN127, HGADFN155, HGADFN164, HGADFN188) sequentially probed with anti-lamin A/C, anti- lamin A, anti-progerin (progerin) and anti-actin antibodies. Right panel: Western blot of protein extracts from skin of individuals at different ages as indicated (49 to 88 Year old, Y denote year). Blots were sequentially probed with antibodies cited above. (B) In situ detection of lamin A/C and Rb in human skin sections. Skin sections from 93-year-old and 38-year-old subjects were probed with antibodies against lamin A/C (red) and Rb (green) and signal overlap is shown at right (Lamin A/C/Rb). (C) Skin sections as in panel (B) were probed with antibodies against lamin A (green) and progerin (red) and DNA was stained with 4′,6-diamidino-2-phenylindole (blue); the merged signals are indicated. Scale bar, 20 µM.

Figure 6

doi: https://doi.org/10.1371/journal.pone.0011132.g006