B-type lamins are fundamental components of the nuclear lamina, a complex structure that acts as a scafold 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 modifcations 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 stifness. 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 fuctuations 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 fndings on the biological role of B-type lamins under physiological or pathological conditions, with a particular emphasis on brain disorders and cancer.

Camilla Evangelisti, I.R. (2022). The wide and growing range of lamin B‑related diseases: from laminopathies to cancer. CELLULAR AND MOLECULAR LIFE SCIENCES, 79(2), 1-11 [10.1007/s00018-021-04084-2].

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

Camilla Evangelisti
Primo
;
Isabella Rusciano;Sara Mongiorgi;Giulia Ramazzotti;Lucia Manzoli
;
Lucio Cocco
;
Stefano Ratti
Ultimo
2022

Abstract

B-type lamins are fundamental components of the nuclear lamina, a complex structure that acts as a scafold 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 modifcations 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 stifness. 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 fuctuations 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 fndings on the biological role of B-type lamins under physiological or pathological conditions, with a particular emphasis on brain disorders and cancer.
2022
Camilla Evangelisti, I.R. (2022). The wide and growing range of lamin B‑related diseases: from laminopathies to cancer. CELLULAR AND MOLECULAR LIFE SCIENCES, 79(2), 1-11 [10.1007/s00018-021-04084-2].
Camilla Evangelisti, Isabella Rusciano, Sara Mongiorgi, Giulia Ramazzotti, Giovanna Lattanzi, Lucia Manzoli, Lucio Cocco, Stefano Ratti
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11585/853971
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