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

Nesprins: from the nuclear envelope and beyond.

Author information

1
James Black Centre, Cardiovascular Division, King's College London, 125 Coldharbour lane, London SE5 9NU, UK.

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.

PMID:
23830188
PMCID:
PMC3733404
DOI:
10.1017/erm.2013.6
[Indexed for MEDLINE]
Free PMC Article

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