Nesprins: from the nuclear envelope and beyond
Open Access
- 5 July 2013
- journal article
- review article
- Published by Cambridge University Press (CUP) in Expert Reviews in Molecular Medicine
- Vol. 15, e5
- https://doi.org/10.1017/erm.2013.6
Abstract
Nuclearenvelopespectrin-repeatproteins(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.Keywords
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