Nesprins: from the nuclear envelope and beyond.

Dipen Rajgor*, Catherine M. Shanahan

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

159 Citations (Scopus)

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.

Original languageEnglish
JournalEXPERT REVIEWS IN MOLECULAR MEDICINE
Volume15
Publication statusPublished - 1 Dec 2013

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