title: Nesprins: from the nuclear envelope and beyond.
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.
authors.affiliation: James Black Centre, Cardiovascular Division, King's College London, 125 Coldharbour lane, London SE5 9NU, UK.
authors.forename: Dipen
authors.initials: D
authors.lastname: Rajgor
authors.forename: Catherine M
authors.initials: CM
authors.lastname: Shanahan
mesh_terms: D000818:Animals; D006801:Humans; D034882:Lamins / Q000378:metabolism; D009685:Nuclear Envelope / Q000378:metabolism*; D009687:Nuclear Proteins / Q000378:metabolism*; D009928:Organ Specificity
chemical_list: D034882:Lamins; D009687:Nuclear Proteins