The wide and growing range of lamin B-related diseases: from laminopathies to cancer.
Brain
LMNB
Lamin B
Laminopathy
Nuclear lamina
Tumour
Journal
Cellular and molecular life sciences : CMLS
ISSN: 1420-9071
Titre abrégé: Cell Mol Life Sci
Pays: Switzerland
ID NLM: 9705402
Informations de publication
Date de publication:
08 Feb 2022
08 Feb 2022
Historique:
received:
20
09
2021
accepted:
08
12
2021
revised:
03
12
2021
entrez:
8
2
2022
pubmed:
9
2
2022
medline:
22
2
2022
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 35132494
doi: 10.1007/s00018-021-04084-2
pii: 10.1007/s00018-021-04084-2
pmc: PMC8821503
doi:
Substances chimiques
Lamin Type B
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
126Informations de copyright
© 2022. The Author(s).
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