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Acknowledgements
This work was supported by the Italian Istituto Superiore di Sanità ‘Rare Diseases Italy-USA program’ [grant number 526/D30], Prin 2008 to G.L., the ‘Fondazione Carisbo’, Italy and A.I.Pro.Sa.B., Italy.
Figures and Tables
Figure 1 Immunochemical analysis of restrictive dermopathy fibroblasts. (A) Low passage cells. CTRL: control; RD: restrictive dermopathy; TEM immunogold labelling, Cyt: cytoplasm; Nu: nucleus; black arrows: gold granules on nuclear lamina; western blot, black arrowheads: prelamin A bands; TEM morphology, white star: dysmorphic nucleus; white arrowheads: nuclear envelope duplications; white square: heterochromatin loss. (B) High passage cells. Immunofluorescence, CTRL: control; RD: restrictive dermopathy; western blot, black arrowheads: prelamin A doublet; TEM morphology: heterochromatin recovery in RD. (C) Immunofluorescence; white arrowheads: normally shaped nuclei; white asterisks: dysmorphic nuclei; western blot, CTRL: control; RD: restrictive dermopathy; NT: not treated with mevinolin; mev: treated with mevinolin; low: low passage cells; high: high passage cells; black arrows: prelamin A bands. (D) IP: immunoprecipitation; white asterisks: prelamin A band position, overlapped by anti-farnesyl antibody; immunofluorescence, RD: restrictive dermopathy. All immunofluorescence pictures are taken at the same magnification.
