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The wide and growing range of lamin B-related diseases: from laminopathies to cancer

Authors :
Camilla Evangelisti
Isabella Rusciano
Sara Mongiorgi
Giulia Ramazzotti
Giovanna Lattanzi
Lucia Manzoli
Lucio Cocco
Stefano Ratti
Camilla Evangelisti, Isabella Rusciano, Sara Mongiorgi, Giulia Ramazzotti, Giovanna Lattanzi, Lucia Manzoli, Lucio Cocco, Stefano Ratti
Source :
Cellular and Molecular Life Sciences. 79
Publication Year :
2022
Publisher :
Springer Science and Business Media LLC, 2022.

Abstract

B-type lamins are fundamental components of the nuclear lamina, a complex structure that acts as a scaffold for organization and function of the nucleus. Lamin B1 and B2, the most represented isoforms, are encoded by LMNB1 and LMNB2 gene, respectively. All B-type lamins are synthesized as precursors and undergo sequential post-translational modifications to generate the mature protein. B-type lamins are involved in a wide range of nuclear functions, including DNA replication and repair, regulation of chromatin and nuclear stiffness. Moreover, lamins B1 and B2 regulate several cellular processes, such as tissue development, cell cycle, cellular proliferation, senescence, and DNA damage response. During embryogenesis, B-type lamins are essential for organogenesis, in particular for brain development. As expected from the numerous and pivotal functions of B-type lamins, mutations in their genes or fluctuations in their expression levels are critical for the onset of several diseases. Indeed, a growing range of human disorders have been linked to lamin B1 or B2, increasing the complexity of the group of diseases collectively known as laminopathies. This review highlights the recent findings on the biological role of B-type lamins under physiological or pathological conditions, with a particular emphasis on brain disorders and cancer. Graphical abstract

Details

ISSN :
14209071 and 1420682X
Volume :
79
Database :
OpenAIRE
Journal :
Cellular and Molecular Life Sciences
Accession number :
edsair.doi.dedup.....b74ae1c32e2f97e9035a6954d0524c67
Full Text :
https://doi.org/10.1007/s00018-021-04084-2