Cellular and Molecular Life Sciences CMLS

, Volume 58, Issue 12, pp 1748-1757

First online:

The structure and function of nuclear lamins: implications for disease

  • R.D. MoirAffiliated withDepartment of Cell and Molecular Biology, Northwestern University Medical School, 303 East Chicago Avenue, Chicago (Illinois 60611, USA), Fax +1 312 503 0954, e-mail: r-moir@northwestern.edu, t-spann@northwestern.edu
  • , T.P. SpannAffiliated withDepartment of Cell and Molecular Biology, Northwestern University Medical School, 303 East Chicago Avenue, Chicago (Illinois 60611, USA), Fax +1 312 503 0954, e-mail: r-moir@northwestern.edu, t-spann@northwestern.edu

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Abstract:

The nuclear lamins polymerize to form the nuclear lamina, a fibrous structure found on the inner face of the nuclear membrane. The lamins also appear to form structures within the nucleoplasm. These various lamin structures help to establish and maintain the shape and strength of the interphase nucleus, but recent work also suggests that the lamins have a role in nuclear processes such as DNA replication. Furthermore, mutations in the human lamin A/C gene have recently been linked to several diseases, including Emery-Dreifuss muscular dystrophy. This review discusses the nature of these mutations and the possible effects of lamin mutations on nuclear function.

Key words: Nuclear lamin; Emery-Dreifuss muscular dystrophy; DNA replication.