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From lamins to lamina: a structural perspective

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Abstract

Lamin proteins are the major constituents of the nuclear lamina, a proteinaceous network that lines the inner nuclear membrane. Primarily, the nuclear lamina provides structural support for the nucleus and the nuclear envelope; however, lamins and their associated proteins are also involved in most of the nuclear processes, including DNA replication and repair, regulation of gene expression, and signaling. Mutations in human lamin A and associated proteins were found to cause a large number of diseases, termed ‘laminopathies.’ These diseases include muscular dystrophies, lipodystrophies, neuropathies, and premature aging syndromes. Despite the growing number of studies on lamins and their associated proteins, the molecular organization of lamins in health and disease is still elusive. Likewise, there is no comprehensive view how mutations in lamins result in a plethora of diseases, selectively affecting different tissues. Here, we discuss some of the structural aspects of lamins and the nuclear lamina organization, in light of recent results.

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Acknowledgments

We thank Reimer Stick for generously providing us with image material. The work is supported by a Swiss National Science Foundation grant (SNSF 31003A_141083/1) to O.M. and a postdoctoral fellowship from the German Academic Exchange Service (DAAD [D/11/44980]) to M.Z.

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  1. Department of Biochemistry, University of Zurich, Winterthurerstrasse 190, 8057, Zurich, Switzerland

    Monika Zwerger & Ohad Medalia

  2. Department of Life Sciences and the National Institute for Biotechnology in the Negev, Ben-Gurion University, 84105, Beer-Sheva, Israel

    Ohad Medalia

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  1. Monika Zwerger
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Correspondence to Ohad Medalia.

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Zwerger, M., Medalia, O. From lamins to lamina: a structural perspective. Histochem Cell Biol 140, 3–12 (2013). https://doi.org/10.1007/s00418-013-1104-y

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