Abstract

The nuclear membrane envelops the nucleoplasm of the cell and acts as a barrier between the nuclear DNA and the cytoplasm. The composition of the nuclear membrane network is complex and incompletely understood, but it provides a structural framework to support and protect the nucleus. Proteins that interact with the nuclear membrane are critical for nuclear stability, especially in striated muscle tissues that undergo intense mechanical stress. One of these proteins in particular, lamin A/C, is known to be involved in at least 11 distinct diseases with variable levels of cardioskeletal involvement. This chapter will explore the nuclear envelope from three perspectives: the function of nuclear proteins on a cellular level, their role in a variety of diseases, and a number of animal models that are being utilized to develop potential therapeutics and treatments.

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