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The Sarcomere and Skeletal Muscle Disease pp 165–175Cite as

Muscular Integrity—A Matter of Interlinking Distinct Structures via Plectin

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Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 642))

Abstract

Myocytes are characterized by the presence of highly specialized cytoskeletal structures that are part of regularly spaced functional units distributed over long distances. In this chapter we discuss previously published evidence as well as novel data showing that the proper positioning and architecture of Z-disks and of sarcolemma-associated costameric structures are largely dependent on the cytolinker protein plectin and its associated intermediate filament (desmin) cytoskeleton. Deficiency in either plectin or desmin lead to muscular dystrophies of similar pathology. However, while in the absence of plectin, desmin networks collapse and form aggregates, when desmin is missing, plectin retains its typical localization. This suggests that plectin recruits and anchors desmin filaments to both Z-disks and costameres and thus is a key element for maintaining and reinforcing myocyte cytoarchitecture. We hypothesize that as an essential link of the Z-disk-costamere axis, plectin is likely to play also a crucial role in myofiber signaling.

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  1. Max F. Perutz Laboratories, Department of Molecular Cell Biology, University of Vienna, A-1030, Vienna, Austria

    Patryk Konieczny & Gerhard Wiche

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  1. Patryk Konieczny
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  2. Gerhard Wiche
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Correspondence to Gerhard Wiche .

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  1. Centre for Medical Research, University of Western Australia, Western Australian Institute for Medical Research, Nedlands, Western Australia, Australia

    Nigel G. Laing PhD

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Konieczny, P., Wiche, G. (2008). Muscular Integrity—A Matter of Interlinking Distinct Structures via Plectin. In: Laing, N.G. (eds) The Sarcomere and Skeletal Muscle Disease. Advances in Experimental Medicine and Biology, vol 642. Springer, New York, NY. https://doi.org/10.1007/978-0-387-84847-1_12

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