Linker of nucleoskeleton and cytoskeleton complex proteins in cardiomyopathy

Biophys Rev. 2018 Aug;10(4):1033-1051. doi: 10.1007/s12551-018-0431-6. Epub 2018 Jun 4.

Abstract

The linker of nucleoskeleton and cytoskeleton (LINC) complex couples the nuclear lamina to the cytoskeleton. The LINC complex and its associated proteins play diverse roles in cells, ranging from genome organization, nuclear morphology, gene expression, to mechanical stability. The importance of a functional LINC complex is highlighted by the large number of mutations in genes encoding LINC complex proteins that lead to skeletal and cardiac myopathies. In this review, the structure, function, and interactions between components of the LINC complex will be described. Mutations that are known to cause cardiomyopathy in patients will be discussed alongside their respective mouse models. Furthermore, future challenges for the field and emerging technologies to investigate LINC complex function will be discussed.

Keywords: Cardiomyocyte; Cardiomyopathy; Inner nuclear membrane; LINC complex; Laminopathy; Nuclear envelope; Nucleus; Outer nuclear membrane.

Publication types

  • Review