The Nuclear Envelope Protein Emerin and Its Interacting Proteins

Abstract

Emerin is a highly conserved and ubiquitously expressed inner nuclear membrane protein of the nuclear envelope. Mutations in the gene encoding emerin cause X‐linked Emery–Dreifuss muscular dystrophy (EDMD), a tissue‐specific, progressive disease that selectively affects skeletal muscle, the cardiac conduction system and tendons. Emerin regulates a number of nuclear functions through interactions with many different binding partners. These emerin‐regulated cellular functions include regulating genomic architecture, maintaining nuclear structure and regulating gene transcription in response to mechanical and chemical signals. Emerin regulation of these activities is important for controlling the coordinated temporal expression of myogenic differentiation genes during skeletal muscle regeneration. This article focuses on the mechanisms by which emerin regulates chromatin architecture and gene transcription and how these mechanisms may function in the development of EDMD.

Key Concepts

  • Proteins of the nuclear lamina are important for chromatin architecture and regulation of gene transcription.
  • Emerin is a nuclear lamina protein that plays key roles in regulating chromatin organisation and gene expression.
  • Emerin regulates the coordinated temporal expression of myogenic differentiation genes.
  • Emerin is required for proper myogenic differentiation.
  • Loss of emerin causes Emery–Dreifuss muscular dystrophy.

Keywords: emerin; lamin; transcription regulation; chromatin architecture; cell signalling; myogenic differentiation; Emery–Dreifuss muscular dystrophy

Figure 1. The nuclear envelope. Emerin, Lap2β and MAN1 bind to lamins at the inner nuclear membrane of the nuclear envelope, where they perform diverse functions within the nucleus. Emerin regulates gene expression by regulating chromatin architecture, binding to transcription factors and altering signalling pathways. ONM, outer nuclear membrane; INM, inner nuclear membrane; ER, endoplasmic reticulum.
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Further Reading

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Collins, Carol M, Nee, Kimbre A, and Holaska, James M(Sep 2016) The Nuclear Envelope Protein Emerin and Its Interacting Proteins. In: eLS. John Wiley & Sons Ltd, Chichester. http://www.els.net [doi: 10.1002/9780470015902.a0025974]