caa a22 4500 475774019 CHVBK 20180406123625.0 cr unu---uuuuu 170329e20000701xx s 000 0 eng 10.1007/s004180000158 doi (NATIONALLICENCE)springer-10.1007/s004180000158 Differences in the distribution of synemin, paranemin, and plectin in skeletal muscles of wild-type and desmin knock-out mice [Elektronische Daten] [Lena Carlsson, Zhen Lin Li, Denise Paulin, Maureen G. Price, Jennifer Breckler, Richard M. Robson, Gerhard Wiche, Lars-Eric Thornell] Abstract.: Mice lacking the gene encoding for the intermediate filament protein desmin have a surprisingly normal myofibrillar organization in skeletal muscle fibers, although myopathy develops in highly used muscles. In the present study we examined how synemin, paranemin, and plectin, three key cytoskeletal proteins related to desmin, are organized in normal and desmin knock-out (K/O) mice. We show that in wild-type mice, synemin, paranemin, and plectin were colocalized with desmin in Z-disc-associated striations and at the sarcolemma. All three proteins were also present at the myotendinous junctions and in the postsynaptic area of motor endplates. In the desmin K/O mice the distribution of plectin was unaffected, whereas synemin and paranemin were partly affected. The Z-disc-associated striations were in general no longer present in between the myofibrils. In contrast, at the myotendinous and neuromuscular junctions synemin and paranemin were still present. Our study shows that plectin differs from synemin and paranemin in its binding properties to the myofibrillar Z-discs and that the cytoskeleton in junctional areas is particularly complex in its organization. Springer-Verlag, 2000 Cytoskeleton Desmin knock-out mice Intermediate filament-associated proteins Myotendinous junctions Neuromuscular junctions nationallicence Carlsson Lena Department of Integrative Medical Biology, Section for Anatomy, Umeå University, 90187 Umeå, Sweden aut Li Zhen Lin Université Paris VII, 2 Place Jussieu, 75005 Paris, France aut Paulin Denise Université Paris VII, 2 Place Jussieu, 75005 Paris, France aut Price Maureen G. Department of Cell Biology, Baylor College of Medicine, Houston, Tex., USA aut Breckler Jennifer Department of Biology, San Francisco State University, San Francisco, Calif., USA aut Robson Richard M. Muscle Biology Group, Departments of Biochemistry, Biophysics and Molecular Biology and of Animal Science, Iowa State University, Ames, Iowa 50011-3260, USA aut Wiche Gerhard Institute of Biochemistry and Molecular Cell Biology, University of Vienna, Vienna Biocenter, 1030 Vienna, Austria aut Thornell Lars-Eric Department of Integrative Medical Biology, Section for Anatomy, Umeå University, 90187 Umeå, Sweden aut https://doi.org/10.1007/s004180000158 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s004180000158 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Carlsson Lena Department of Integrative Medical Biology, Section for Anatomy, Umeå University, 90187 Umeå, Sweden aut NATIONALLICENCE 700 1- Li Zhen Lin Université Paris VII, 2 Place Jussieu, 75005 Paris, France aut NATIONALLICENCE 700 1- Paulin Denise Université Paris VII, 2 Place Jussieu, 75005 Paris, France aut NATIONALLICENCE 700 1- Price Maureen G. Department of Cell Biology, Baylor College of Medicine, Houston, Tex., USA aut NATIONALLICENCE 700 1- Breckler Jennifer Department of Biology, San Francisco State University, San Francisco, Calif., USA aut NATIONALLICENCE 700 1- Robson Richard M. Muscle Biology Group, Departments of Biochemistry, Biophysics and Molecular Biology and of Animal Science, Iowa State University, Ames, Iowa 50011-3260, USA aut NATIONALLICENCE 700 1- Wiche Gerhard Institute of Biochemistry and Molecular Cell Biology, University of Vienna, Vienna Biocenter, 1030 Vienna, Austria aut NATIONALLICENCE 700 1- Thornell Lars-Eric Department of Integrative Medical Biology, Section for Anatomy, Umeå University, 90187 Umeå, Sweden aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer