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Published online March 26, 2007
doi:10.1083/jcb.200604179
The Journal of Cell Biology, Vol. 176, No. 7, 965-977
The Rockefeller University Press, 0021-9525 $30.00
© 2007 Rezniczek et al.
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Article

 Plectin 1f scaffolding at the sarcolemma of dystrophic (mdx) muscle fibers through multiple interactions with ß-dystroglycan

Günther A. Rezniczek1, Patryk Konieczny1, Branislav Nikolic1, Siegfried Reipert1, Doris Schneller1, Christina Abrahamsberg1, Kay E. Davies2, Steve J. Winder3, and Gerhard Wiche1

1 Max F. Perutz Laboratories, Department of Molecular Cell Biology, University of Vienna, A-1030 Vienna, Austria
2 Department of Physiology, Anatomy, and Genetics, Medical Reasearch Council Functional Genetics Unit, Oxford OX1 3QX, England, UK
3 Centre for Developmental and Biomedical Genetics, Department of Biomedical Science, University of Sheffield, Western Bank, Sheffield S10 2TN, England, UK

Correspondence to Gerhard Wiche: gerhard.wiche{at}univie.ac.at

In skeletal muscle, the cytolinker plectin is prominently expressed at Z-disks and the sarcolemma. Alternative splicing of plectin transcripts gives rise to more than eight protein isoforms differing only in small N-terminal sequences (5–180 residues), four of which (plectins 1, 1b, 1d, and 1f) are found at substantial levels in muscle tissue. Using plectin isoform–specific antibodies and isoform expression constructs, we show the differential regulation of plectin isoforms during myotube differentiation and their localization to different compartments of muscle fibers, identifying plectins 1 and 1f as sarcolemma-associated isoforms, whereas plectin 1d localizes exclusively to Z-disks. Coimmunoprecipitation and in vitro binding assays using recombinant protein fragments revealed the direct binding of plectin to dystrophin (utrophin) and ß-dystroglycan, the key components of the dystrophin–glycoprotein complex. We propose a model in which plectin acts as a universal mediator of desmin intermediate filament anchorage at the sarcolemma and Z-disks. It also explains the plectin phenotype observed in dystrophic skeletal muscle of mdx mice and Duchenne muscular dystrophy patients.

Abbreviations used in this paper: ABD, actin-binding domain; ßDG, ß-dystroglycan; DGC, dystrophin–glycoprotein complex; DMD, Duchenne MD; EDL, extensor digitorum longus; IB, immunoblotting; IF, intermediate filament; IFM, immunofluorescence microscopy; IP, immunoprecipitation; MD, muscular dystrophy; MyHC, myosin heavy chain.


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