Good news in the nuclear envelope: loss of lamin A might be a gain

The Journal of Clinical Investigation
Paola Scaffidi, T Misteli

Abstract

Genetic diseases often reveal the physiological roles of the affected proteins. The identification of mutations in the nuclear envelope proteins lamin A and lamin C as the cause of a diverse group of human diseases has expanded our understanding of the lamin proteins from being merely structural elements of the cell nucleus and has implicated them in novel cellular functions including signal transduction and gene expression. However, it now appears that the physiological relevance of one of the lamin proteins in organismal function has been overestimated. In this issue of the JCI, Fong et al. demonstrate that lamin A-deficient mice are phenotypically normal (see the related article beginning on page 743). The good news is these findings open the door to a new strategy for the therapeutic treatment of diseases caused by mutations in lamin A, such as muscular dystrophies and some types of premature aging syndromes.

References

Sep 18, 2002·Proceedings of the National Academy of Sciences of the United States of America·Martin O BergoStephen G Young
Apr 19, 2003·Science·Annachiara De Sandre-GiovannoliNicolas Lévy
Jun 6, 2003·Current Opinion in Cell Biology·Dale K ShumakerRobert D Goldman
Feb 3, 2004·The Journal of Clinical Investigation·Jan LammerdingRichard T Lee
Oct 21, 2004·The Journal of Pathology·Jos L V BroersFrans C S Ramaekers
Mar 3, 2006·The Journal of Clinical Investigation·Loren G FongStephen G Young

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Citations

Feb 16, 2007·The Journals of Gerontology. Series A, Biological Sciences and Medical Sciences·Julius Halaschek-Wiener, Angela Brooks-Wilson
Apr 28, 2010·Human Molecular Genetics·Brandon S J DaviesLoren G Fong
Sep 5, 2008·The Journal of Clinical Investigation·Shao H YangLoren G Fong
Jun 15, 2011·The Journal of Clinical Investigation·Kan CaoFrancis S Collins
Mar 27, 2013·The Journal of Cell Biology·Daniel W BusterGregory C Rogers
Jun 22, 2016·Current Molecular Biology Reports·Gunes UzerJanet Rubin
Apr 9, 2008·European Journal of Cell Biology·Gabriela GaliováStanislav Kozubek
Apr 5, 2011·Acta Biochimica Et Biophysica Sinica·Haibo WangCheng-Wu Chi

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Methods Mentioned

BETA
antisense oligonucleotides

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