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Journal of Biomedicine and Biotechnology
Volume 2011 (2011), Article ID 569628, 12 pages
http://dx.doi.org/10.1155/2011/569628
Review Article

The Sarcomeric Z-Disc and Z-Discopathies

Centre for Research Excellence, British Heart Foundation, National Heart & Lung Institute, Imperial College, South Kensington Campus, Flowers Building, 4th Floor, London SW7 2AZ, UK

Received 5 July 2011; Accepted 12 August 2011

Academic Editor: Henk Granzier

Copyright © 2011 Ralph Knöll et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

The sarcomeric Z-disc defines the lateral borders of the sarcomere and has primarily been seen as a structure important for mechanical stability. This view has changed dramatically within the last one or two decades. A multitude of novel Z-disc proteins and their interacting partners have been identified, which has led to the identification of additional functions and which have now been assigned to this structure. This includes its importance for intracellular signalling, for mechanosensation and mechanotransduction in particular, an emerging importance for protein turnover and autophagy, as well as its molecular links to the t-tubular system and the sarcoplasmic reticulum. Moreover, the discovery of mutations in a wide variety of Z-disc proteins, which lead to perturbations of several of the above-mentioned systems, gives rise to a diverse group of diseases which can be termed Z-discopathies. This paper provides a brief overview of these novel aspects as well as points to future research directions.