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The Lamin Proteins in Nuclear Structure, Functions, and Laminopathies
Gan Zhao1, Ziheng Chen1, Caifeng Yang1
1The Academy for Cell and Life Health, Faculty of Life Science and Technology, Kunming University of Science and Technology, Kunming 650500, China.
Cells
|June 25, 2026
Summary
Lamin proteins form the nuclear lamina, essential for nuclear structure and function. Mutations cause diverse laminopathies, highlighting lamins
Area of Science:
- Cell Biology
- Molecular Biology
- Genetics
Background:
- Lamin proteins (A- and B-types) and associated proteins constitute the nuclear lamina.
- The nuclear lamina regulates nuclear mechanics, chromatin organization, and gene regulation.
- Mutations in lamin proteins cause a spectrum of tissue-specific disorders known as laminopathies.
Purpose of the Study:
- To elucidate the functional roles of lamin proteins beyond structural support.
- To understand the tissue-specific mechanisms underlying laminopathies.
- To explore therapeutic strategies for laminopathies.
Main Methods:
- Analysis of patient-derived cellular models.
- Investigation using animal systems.
- Review of molecular and cellular mechanisms of lamin function and dysfunction.
Main Results:
- Lamins act as active regulators: A-type lamins influence nuclear stiffness and gene transcription, while B-type lamins ensure mechanical resilience.
- Pathogenic mutations disrupt lamin functions via convergent mechanisms, leading to tissue-specific pathologies.
- Disruptions manifest as impaired nuclear mechanics, aberrant gene regulation, defective DNA repair, and cellular senescence.
Conclusions:
- Lamin proteins are crucial for maintaining nuclear integrity and function.
- Laminopathies arise from tissue-specific consequences of nuclear dysfunction.
- Advances in modeling and understanding vulnerabilities pave the way for novel therapeutic interventions.
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