Journal
CURRENT OPINION IN CELL BIOLOGY
Volume 54, Issue -, Pages 72-79Publisher
CURRENT BIOLOGY LTD
DOI: 10.1016/j.ceb.2018.05.001
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Funding
- Swiss National Science Foundation [SNSF 31003A_179418/1]
- Maxi Foundation [GIF I-1289-412.13/2015]
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Lamins are the main component of the nuclear lamina, a protein meshwork at the inner nuclear membrane which primarily provide mechanical stability to the nucleus. Lamins, type V intermediate filament proteins, are also involved in many nuclear activities. Structural analysis of nuclei revealed that lamins form 3.5 nm thick filaments often interact with nuclear pore complexes. Mutations in the LMNA gene, encoding A-type lamins, have been associated with at least 15 distinct diseases collectively termed laminopathies, including muscle, metabolic and neurological disorders, and premature aging syndrome. It is unclear how laminopathic mutations lead to such a wide array of diseases, essentially affecting almost all tissues.
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