Nesprins: from the nuclear envelope and beyond

Expert Rev Mol Med. 2013 Jul 5;15:e5. doi: 10.1017/erm.2013.6.

Abstract

Nuclear envelope spectrin-repeat proteins (Nesprins), are a novel family of nuclear and cytoskeletal proteins with rapidly expanding roles as intracellular scaffolds and linkers. Originally described as proteins that localise to the nuclear envelope (NE) and establish nuclear-cytoskeletal connections, nesprins have now been found to comprise a diverse spectrum of tissue specific isoforms that localise to multiple sub-cellular compartments. Here, we describe how nesprins are necessary in maintaining cellular architecture by acting as essential scaffolds and linkers at both the NE and other sub-cellular domains. More importantly, we speculate how nesprin mutations may disrupt tissue specific nesprin scaffolds and explain the tissue specific nature of many nesprin-associated diseases, including laminopathies.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Humans
  • Lamins / metabolism
  • Nuclear Envelope / metabolism*
  • Nuclear Proteins / metabolism*
  • Organ Specificity

Substances

  • Lamins
  • Nuclear Proteins