Table 1.

Consequences of lamin mutations on nuclear structure and mechanics

Evidence of altered nuclear stability	Experimental system	References
Nuclear rupture	In vitro: in patient fibroblasts carrying mutations that lead to HGPS, FPLD, RD and HCM In vivo: in the heart tissue of human and mouse carrying DCM mutations	[41] [48,49]
Stiffer nuclei	In vitro: in HGPS patient cells and in Xenopus oocytes overexpressing lamin A with a mutation that causes HGPS	[19,50]
Softer nuclei	In vitro: in fibroblasts carrying mutations that lead to DCM and EDMD In situ: In Drosophila larval muscle expressing dominant-negative lamin mutations	[43]
Elongated nuclei in vivo	In the heart tissue of human, mice, and flies carrying DCM mutations	[43–47]
Defects in lamin assembly	In vitro assembly of lamin filaments and paracrystals from recombinant lamin	[43,56–59]