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Difference in the expression pattern of dystrophin on the surface membrane between the skeletal and cardiac muscles of mdx carrier mice

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Summary

We examined the expression of dystrophin by immunohistochemical and immunoblot analyses in the skeletal and cardiac muscles of Xmdx/X+ heterozygous mice, which were obtained by mating male mdx mice (Xmdx/Y) with female wild type mice (X+/X+). Dystrophin was expressed on the surface membrane in both muscles, but the mode of expression was different between the two muscles. In cardiac muscle, dystrophin positive and negative cells were present in roughly equal numbers intermingled in a mosaic pattern; this was considered to reflect the random inactivation of X-chromosomes in early development. In skeletal muscle, most of the surface membrane was dystrophin positive. There were little signs of fiber necrosis or regeneration, and serum creatine kinase levels were normal. We are at present of opinion that the predominance of dystrophin-positive area in skeletal muscle is due to intracellular diffusion of dystrophin.

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References

  • Arahata K, Ishiura S, Ishiguro T, Tsukahara T, Suhara Y, Eguchi C, Ishihara T, Nonaka I, Ozawa E, Sugita H (1988) Immunostaining of skeletal and cardiac surface membrane with antibody against Duchenne muscular dystrophy peptide. Nature 333:861–863

    Article  PubMed  CAS  Google Scholar 

  • Arahata K, Ishihara T, Kamakura K, Tsukahara T, Ishiura S, Baba C, Matsumoto T, Nonaka I, Sugita H (1989a) Dystrophin and Duchenne's muscular dystrophy. Nature 321:398–399

    Google Scholar 

  • Arahata K, Hoffman EP, Kunkel LM, Ishiura S, Tsukahara T, Ishihara T, Sunohara N, Nonaka I, Ozawa E, Sugita H (1989b) Dystrophin diagnosis: comparison of dystrophin abnormalities by immunofluorescence and immunoblot analyses. Proc Natl Acad Sci USA 86:7154–7158

    Article  PubMed  CAS  Google Scholar 

  • Bonilla E, Samitt CE, Miranda AF, Hays AP, Salviati IG, Di-Mauro S, Kunkel LM, Hoffman EP, Rowland LP (1988) Duchenne muscular dystrophy: deficiency of dystrophin at the muscle cell surface. Cell 54:447–452

    Article  PubMed  CAS  Google Scholar 

  • Cooper AR, Kurkinen M, Taylor A, Hogan BLM (1981) Studies on the biosynthesis of laminin by murine parietal endoderm cells. Eur J Biochem 119:189–197

    Article  PubMed  CAS  Google Scholar 

  • Frair PM, Peterson AC (1983) The nuclear-cytoplasmic relationship in ‘mosaic’ skeletal muscle fibers from mouse chimeras. Exp Cell Res 145:167–178

    Article  PubMed  CAS  Google Scholar 

  • Gartler SM, Riggs AD (1983) Mammalian X-chromosome inactivation. Ann Rev Genet 17:155–190

    Article  PubMed  CAS  Google Scholar 

  • Hagiwara Y, Yoshida M, Nonaka I, Ozawa E (1989) Developmental expression of dystrophin on the plasma membrane of rat muscle cells. Protoplasma 151:11–18

    Article  Google Scholar 

  • Hoffman EP, Brown RH Jr, Kunkel LM (1987a) Dystrophin: the protein product of the Duchenne muscular dystrophy gene. Cell 51:919–928

    Article  PubMed  CAS  Google Scholar 

  • Hoffman EP, Kundson CM, Campbell KP, Kunkel LM (1987b) Subcellular fractionation of dystrophin to the triad of skeletal muscle. Nature 330:754–758

    Article  PubMed  CAS  Google Scholar 

  • Hoffman EP, Fischbeck KH, Brown RH, Johnson M, Medori R, Loike JD, Harris JB, Waterston R, Brooke M, Specht L, Kupsky W, Chamberlain J, Caskey CT, Shapiro F, Kunkel LM (1988) Characterization of dystrophin in muscle biopsy specimens from patients with Duchenne's or Becker's muscular dystrophy. N England J Med 318:1363–1368

    Article  CAS  Google Scholar 

  • Hurko O, McKee L, Zuurveld J, Swick HM (1987) Comparison of Duchenne and normal myoblasts from a heterozygote. Neurology 37:675–681

    PubMed  CAS  Google Scholar 

  • Ishikawa H (1966) Electron microscopic observations of satellite cells with special reference to the development of mammalian skeletal muscles. Z Anat Entwicklungsgesch 125:43–63

    Article  PubMed  CAS  Google Scholar 

  • Koenig M, Monaco AP, Kunkel LM (1988) The complete sequence of dystrophin predicts a rod-shaped cytoskeletal protein. Cell 53:219–228

    Article  PubMed  CAS  Google Scholar 

  • Kundson CM, Hoffman EP, Kahl SD, Kunkel LM, Campbell KP (1988) Evidence for the association of dystrophin with the transverse tubular system in skeletal muscle. J Biol Chem 263:8480–8484

    Google Scholar 

  • Lev AA, Feener CC, Kunkel LM, Brown RH Jr (1987) Expression of the Duchenne's muscular dystrophy gene in cultured muscle cells. J Biol Chem 262:15817–15820

    PubMed  CAS  Google Scholar 

  • Love DR, Hill DF, Dickson G, Spurr NK, Byth BC, Marsden RF, Walsh FS, Edwards YH, Davies KE (1989) An autosomal transcript in skeletal muscle with homology to dystrophin. Nature 339:55–58

    Article  PubMed  CAS  Google Scholar 

  • Lyon MF (1961) Gene action in the X-chromosome of the mouse (Mus musculus L.). Nature 190:372–373

    Article  PubMed  CAS  Google Scholar 

  • Lyon MF (1972) X-chromosome inactivation and developmental patterns in mammals. Biol Rev 47:1–35

    Article  PubMed  CAS  Google Scholar 

  • Monc M, Harper MI (1979) Sequential X-chromosome inactivation coupled with cellular differentiation in early mouse embryos. Nature 281:311–313

    Article  Google Scholar 

  • Moss FP, Leblond CP (1971) Satellite cells as the source of nuclei in muscles of growing rats. Anat Rec 170:421–436

    Article  PubMed  CAS  Google Scholar 

  • Nudel U, Robzyc K, Yaffe D (1988) Expression of the putative Duchenne muscular dystrophy gene in differentiated myogenic cell cultures and in the brain. Nature 331:635–638

    Article  PubMed  CAS  Google Scholar 

  • Oronzi Scott M, Sylvester JE, Heilman-Patterson T, Shi Y-J, Fieles W, Stedman H, Burghes A, Ray P, Worton R, Fischbeck KH (1988) Duchenne muscular dystrophy gene expression in normal and diseased human muscle. Science 239:1418–1420

    Article  Google Scholar 

  • Scinski P, Geng Y, Ryder-Cook AS, Barnard EA, Darlison MG, Barnard PJ (1989) The molecular basis of muscular dystrophy in the mdx mouse: a point mutation. Science 244:1578–1580

    Article  Google Scholar 

  • Shimizu T, Matsumura K, Hashimoto K, Mannen T, Ishiguro T, Eguchi C, Nonaka I, Yoshida M, Ozawa E (1988) A monoclonal antibody to a synthetic polypeptide fragment of dystrophin (amino acid sequence from position 215 to 264). Proc Jpn Acad 64b:205–208

    Google Scholar 

  • Stockdale FE, Holtzer H (1961) DNA synthesis and myogenesis. Exp Cell Res 24:508–520

    Article  PubMed  CAS  Google Scholar 

  • Sugita H, Arahata K, Ishiguro T, Suhara Y, Tsukahara T, Ishiura S, Eguchi C, Nonaka I, Ozawa E (1988) Negative immunostaining of Duchenne muscular dystrophy (DMD) and mdx muscle surface membrane with antibody against synthetic peptide fragment predicted from DMD cDNA. Proc Jpn Acad 64b:37–39

    Google Scholar 

  • Tanabe Y, Esaki K, Nomura T (1986) Skeletal muscle pathology in X-chromosome linked muscular dystrophy (mdx) mouse. Acta Neuropathol (Berl) 69:91–95

    Article  PubMed  CAS  Google Scholar 

  • Tanaka H, Yoshida M, Ishiguro T, Eguchi C, Nonaka I, Ozawa E (1989a) Expression of dystrophin of the cell surface membrane of intrafusal fibers of human skeletal muscle. Protoplasma (in press)

  • Tanaka H, Shimizu T, Ozawa E (1989b) Expression of a dystrophin-like protein on the surface membrane of skeletal muscle fibers in mdx mice. Proc Jpn Acad 65 (B) 238–241

    CAS  Google Scholar 

  • Watkins SC, Hoffman EP, Slayter HS, Kunkel LM (1988) Immunoelectron microscopic localization of dystrophin in myoblasts. Nature 333:863–866

    Article  PubMed  CAS  Google Scholar 

  • Zubrzycka-Gaarn EE, Bulman DE, Karpati G, Burghes AHM, Belfall B, Klamut HJ, Talbot J, Hodges RS, Ray PN, Worton RG (1988) The Duchenne muscular dystrophy gene product is localized in sarcolemma of human skeletal muscle. Nature 333:466–469

    Article  PubMed  CAS  Google Scholar 

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On leave from The Department of Pediatrics, Tokyo Women's Medical College

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Tanaka, H., Ikeya, K. & Ozawa, E. Difference in the expression pattern of dystrophin on the surface membrane between the skeletal and cardiac muscles of mdx carrier mice. Histochemistry 93, 447–452 (1990). https://doi.org/10.1007/BF00266399

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