Figure 2

Sub-expression and mislocalization of LAP1. A. Three bands were observed using anti-LAP1 antibody in control fibroblasts. Fibroblasts from the patient had significantly less of the larger isoforms, whereas the expression of the shorter isoform was less affected (LAP1 antibodies: courtesy of Dr. W.T. Dauer, anti-GAPDH: Santa Cruz Biotechnology; Olympus FV-1000 confocal microscope). B. Fibroblasts from patient show a strong reduction or total absence of LAP1 at the nuclear envelope. A faint staining for LAP1 was observed at the ER (stained with calnexin antibodies, Santa Cruz Biotechnology; secondary Alexa-conjugates antibodies, Life Technologies; HRP-conjugated antibodies: Jackson ImmunoResearch), and strong staining was observed in some regions (see magnification in the inset). Scale bar: 10 μm. C. Electron micrograph of patient fibroblast did not reveal alterations at the nuclear envelope. Scale bar: 5 μm; inset: 500 nm. Imaging as described before [3].