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Cited by 391Nuclear Structure and FunctionSkin and Cellular Biology ResearchCellular Mechanics and Interactions

Lamin B1 is required for mouse development and nuclear integrity

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Vergnes, Laurent, Péterfy, Miklós, Bergo, Martin O., Young, Stephen G., Reue, Karen·2004·Proceedings of the National Academy of Sciences
DOI 10.1073/pnas.0401424101PMC PMC478588
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Abstract

Lamins are key structural components of the nuclear lamina, an intermediate filament meshwork that lies beneath the inner nuclear membrane. Lamins play a role in nuclear architecture, DNA replication, and gene expression. Mutations affecting A-type lamins have been associated with a variety of human diseases, including muscular dystrophy, cardiomyopathy, lipodystrophy, and progeria, but mutations in B-type lamins have never been identified in humans or in experimental animals. To investigate the in vivo function of lamin B1, the major B-type lamin, we generated mice with an insertional mutation in Lmnb1. The mutation resulted in the synthesis of a mutant lamin B1 protein lacking several key functional domains, including a portion of the rod domain, the nuclear localization signal, and the CAAX motif (the carboxyl-terminal signal for farnesylation). Homozygous Lmnb1 mutant mice survived embryonic development but died at birth with defects in lung and bone. Fibroblasts from mutant embryos grew under standard cell-culture conditions but displayed grossly misshapen nuclei, impaired differentiation, increased polyploidy, and premature senescence. Thus, the lamin B1 mutant mice provide evidence for a broad and nonredundant function of lamin B1 in mammalian development. These mutant mice and cell lines derived from them will be useful models for studying the role of the nuclear lamina in various cellular processes.

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Authors
Vergnes, L., Péterfy, M., Bergo, M. O., Young, S. G., & Reue, K.
Affiliations
  • University of California, Los Angeles (US)
  • Veterans Affairs Greater Los Angeles Healthcare System and Departments of Medicine and Human Genetics, David Geffen School of Medicine, University of California, Los Angeles, CA 90073; and The Gladstone Institute of Cardiovascular Disease and Department of Medicine, University of California, San Francisco, CA 94141
  • Gladstone Institutes (US)
Corresponding author
Reue, Karen
ORCID
Venue
Proceedings of the National Academy of Sciences
Publisher
National Academy of Sciences
Published
2004-07
Volume
101
Issue
28
Pages
10428-10433
ISSN
  • 0027-8424 (ISSN-L)
  • 1091-6490
DOI
10.1073/pnas.0401424101
PMC
PMC478588
OpenAlex
W2042168939
Cited by
391
References
42
Citations by year
2026: 12 · 2025: 17 · 2024: 16 · 2023: 14 · 2022: 22 · 2021: 23 · 2020: 18 · 2019: 15 · 2018: 15 · 2017: 13 · 2016: 17 · 2015: 12 · 2014: 17 · 2013: 17 · 2012: 21
FWCI
5.53
Type
journal article
Language
en
Open access
Locations
  • Proceedings of the National Academy of Sciences (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
Funders
National Heart, Lung, and Blood Institute (HL-66621, U01HL066621)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Nuclear Structure and Function
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Skin and Cellular Biology Research
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Cellular Mechanics and Interactions
Keywords
lamin B1, nuclear lamina, B-type lamins, mouse development, nuclear integrity, nuclear morphology, polyploidy, premature senescence, fibroblast differentiation, CAAX motif, farnesylation, nuclear localization signal, rod domain, embryonic lethality, bone defects, laminopathies
MeSH terms
Animals, Bone and Bones, Cells, Cultured, Embryonic and Fetal Development, Fibroblasts, Genes, Lethal, Lung, Ossification, Heterotopic, Stem Cells, Mutagenesis, Insertional, Mice, Knockout, Gene Expression Regulation, Developmental, Nuclear Lamina, Lamin Type B, Mice

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