Fig. 1.

A unique Ab specifically detects the mutant lamin A G608G (progerin) in HGPS cells and demonstrates the accumulation of the mutant protein progerin over time. (A) Immunofluorescence microscopy was performed on fibroblasts from an unaffected individual (40 PPDs) and a subject with HGPS (24, 30, 42, and 52 PPDs). Cells were stained with anti-LMNA G608G (red) and anti-emerin (green); the merged signals are indicated. Note the increase of positively labeled HGPS with increasing PPDs. (B) Western blot analysis of control (38 PPDs) and HGPS (28, 35, and 45 PPDs) extracts probed with anti-LMNA G608G and anti-lamin A/C. The arrow indicates the progerin band. (C) HGPS and control fibroblasts double-labeled with anti-LMNA G608G (red) and either anti-lamin A, anti-emerin, or anti-nucleoporin 414 (green) reveal the similar distribution into cable-like structures of mutant and wild-type proteins. Chromatin was stained with DAPI (blue).