caa a22 4500 475744845 CHVBK 20180406123510.0 cr unu---uuuuu 170329e20000801xx s 000 0 eng 10.1023/A:1026587530656 doi (NATIONALLICENCE)springer-10.1023/A:1026587530656 p0071, a member of the armadillo multigene family, is a constituent of sarcomeric I-bands in human skeletal muscle [Elektronische Daten] [Rolf Schröder, Peter Van der Ven, Irene Warlo, Heike Schumann, Dieter Fürst, Ingmar Blümcke, Matthias Schmidt, Mechthild Hatzfeld] p0071 is a member of the armadillo gene family that is expressed in a wide variety of mammalian tissues and cell types with a prominent cell-cell contact association in epithelial cells. Here, we report the expression and localization patterns of p0071 in differentiating human skeletal muscle cells and in normal and diseased human skeletal muscle tissues. Northern blots revealed expression of p0071 mRNA in adult skeletal muscle tissue. RT-PCR analysis and Western blotting experiments identified two differentially spliced isoforms of p0071. The balance between these isoforms shifted during in vitro differentiation of isolated muscle cells from predominant expression of the short variant to a preponderance of the larger variant from day 6 onwards. Immunolocalization studies in mature skeletal muscle tissue revealed that p0071 is a constituent of myofibrils with a distinct localization at the level of sarcomeric N2-lines. During myofibrillogenesis, p0071 was not detected in non-striated nascent myofibrils, but became apparent shortly after the development of compact Z-discs in early myotubes. Furthermore, we studied the expression of p0071 in a wide variety of neuromuscular disorders by indirect immunofluorescence. Here, the myofibrillar staining of p0071 was preserved in all the disease entities included in our study. Our results provide the first evidence that a member of the armadillo multigene family is a constituent of the contractile apparatus in human skeletal muscle. The localization of p0071 at the level of I-bands and the timepoint of its integration into developing myofibrils suggest a possible role in the organization of thin filaments. Kluwer Academic Publishers, 2000 Schröder Rolf Department of Neurology, University Hospital Bonn, Bonn, Germany aut Van der Ven Peter Department of Cell Biology, University of Potsdam, Potsdam, Germany aut Warlo Irene Department of Neuropathology, University Hospital Mainz, Mainz, Germany aut Schumann Heike Molecular Biology Group, Medical Faculty, University of Halle, Halle, Germany aut Fürst Dieter Department of Cell Biology, University of Potsdam, Potsdam, Germany aut Blümcke Ingmar Department of Neuropathology, University Hospital Mainz, Mainz, Germany aut Schmidt Matthias Department of Neurosurgery, University Hospital Bonn, Bonn, Germany aut Hatzfeld Mechthild Molecular Biology Group, Medical Faculty, University of Halle, Halle, Germany aut Journal of Muscle Research & Cell Motility Kluwer Academic Publishers 21/6(2000-08-01), 577-586 0142-4319 21:6<577 2000 21 10974 https://doi.org/10.1023/A:1026587530656 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1023/A:1026587530656 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Schröder Rolf Department of Neurology, University Hospital Bonn, Bonn, Germany aut NATIONALLICENCE 700 1- Van der Ven Peter Department of Cell Biology, University of Potsdam, Potsdam, Germany aut NATIONALLICENCE 700 1- Warlo Irene Department of Neuropathology, University Hospital Mainz, Mainz, Germany aut NATIONALLICENCE 700 1- Schumann Heike Molecular Biology Group, Medical Faculty, University of Halle, Halle, Germany aut NATIONALLICENCE 700 1- Fürst Dieter Department of Cell Biology, University of Potsdam, Potsdam, Germany aut NATIONALLICENCE 700 1- Blümcke Ingmar Department of Neuropathology, University Hospital Mainz, Mainz, Germany aut NATIONALLICENCE 700 1- Schmidt Matthias Department of Neurosurgery, University Hospital Bonn, Bonn, Germany aut NATIONALLICENCE 700 1- Hatzfeld Mechthild Molecular Biology Group, Medical Faculty, University of Halle, Halle, Germany aut NATIONALLICENCE 773 0- Journal of Muscle Research & Cell Motility Kluwer Academic Publishers 21/6(2000-08-01), 577-586 0142-4319 21:6<577 2000 21 10974 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer