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Electron spin resonance studies of an animal model of human congenital myotonia: Increased erythrocyte membrane fluidity in rats with 20,25-diazacholesterol-induced myotonia

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Summary

Electron spin resonance experiments have been performed on erythrocyte membranes from rats with myotonia induced by treatment with 20,25-diazacholesterol. The results suggest that erythrocyte membranes in this animal model of human congenital myotonia possess a highly significantly increased surface membrane fluidity compared to that of controls. Alterations in the physical state of membrane proteins were not apparent. These findings, also present in human congenital myotonia [Butterfield, Chesnut, Roses & Appel, 1976,Nature (London)263:159; Butterfield, 1977 (Submitted for publication)], strengthen the concepts that increased membrane fluidity is associated with the presence of myotonia and that congenital myotonia may be a diffuse membrane disease.

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References

  1. Adams, M.J., Thompson, J.W. 1965. Myotonia congenita in goats: An electron microscopic, biochemical, and biophysical study.Sth. Med. J. 58:1586

    Google Scholar 

  2. Adams, R.D., Denny-Brown, D., Pearson, C.M. 1965. Diseases of Muscle, Second edition. Harper and Row, New York

    Google Scholar 

  3. Brody, I.A. 1974. Sarcolemma defects in myotonia: Animal models and pharmacology.In: Models of Human Neurological Diseases. H.L. Klawans, Jr., editor. Excerpta Medica, Amsterdam

    Google Scholar 

  4. Brown, G.L., Harvey, A.M. 1939. Congenital myotonia in the goat.Brain 62:341

    Google Scholar 

  5. Brown, H.D., Chattopadhyay, S.K., Patel, A.B. 1968. Diazacholesterol effect upon membrane ATPase.Metabolism 17:555

    Article  PubMed  Google Scholar 

  6. Brownlee, K.A. 1960. Statistical Theory and Methodology in Science and Engineering. John Wiley and Sons, New York

    Google Scholar 

  7. Butterfield, D.A., Chesnut, D.B., Roses, A.D., Appel, S.H. 1974. Electron spin resonance studies of erythrocyte membranes from patients with myotonic muscular dystrophy.Proc. Nat. Acad. Sci. USA 71:909

    PubMed  Google Scholar 

  8. Butterfield, D.A., Chesnut, D.B., Roses, A.D., Appel, S.H. 1976. Spin label study of erythrocyte membrane fluidity in myotonic and Duchenne muscular dystrophy and congenital myotonia.Nature (London) 263:159

    Google Scholar 

  9. Butterfield, D.A., Roses, A.D., Appel, S.H., Chesnut, D.B. 1976. Electron spin resonance studies of membrane proteins in erythrocytes in myotonic muscular dystrophy.Arch. Biochem. Biophys. 177:226

    Article  PubMed  Google Scholar 

  10. Butterfield, D.A., Roses, A.D., Cooper, M.L., Appel, S.H., Chesnut, D.B. 1974. A comparative electron spin resonance study of the erythrocyte membrane in myotonic muscular dystrophy.Biochemistry 13:5078

    PubMed  Google Scholar 

  11. Butterfield, D.A., Whisnant, C.C., Chesnut, D.B. 1976. On the use of the spin labeling technique in the study of erythrocyte membranes.Biochim. Biophys. Acta 426:697

    PubMed  Google Scholar 

  12. Chapman, D., Barratt, D.D., Kamat, V.B. 1969. A spin label study of erythrocyte membranes.Biochim. Biophys. Acta 173:154

    PubMed  Google Scholar 

  13. Conrad, J.T., Glaser, G.H. 1961. Bioelectric properties of dystrophic mammalian muscle. Excitability, membrane resting, and action potentials in mouse hereditary myopathy.Arch. Neurol. 5:46

    PubMed  Google Scholar 

  14. Dubowitz, V., Brook, M.H. 1973. Muscle Biopsy: A Modern Approach. W.B. Saunders, Philadelphia

    Google Scholar 

  15. Fairbanks, G., Steck, T.L., Wallach, D.F.H. 1971. Electrophoretic analysis of the major polypeptides of the human erythrocyte membrane.Biochemistry 10:2606

    PubMed  Google Scholar 

  16. Heene, R. 1968. Electron microscopic findings in 2,4-dichlorophenoxyacetate(2,4-D) induced myopathies in mammals. On the development of early myopathic changes.Dtsch. Z. Nervenheilk 193:265

    Article  Google Scholar 

  17. Hofer, S., Hofer, H.W., Kuhn, E., Pette, D. 1971. Changes of hexokinase in the enzyme activity pattern of muscle in human myotonia congenita and in experimental myotonia of the rat.Klin. Wschr. 49:968

    Article  PubMed  Google Scholar 

  18. Holmes, D.E., Piette, L.H. 1970. Effects of phenothiazine derivatives on biological membranes: Drug induced changes in electron spin responance spectra from spin-labeled erythrocyte ghost membranes.J. Pharmacol. Exp. Ther. 173:78

    PubMed  Google Scholar 

  19. Hsia, J.C., Long, R.A., Hruska, F.E., Gesser, H.D. 1972. Steroid-phosphatidyloholine interactions in oriented multibilayers—A spin label study.Biochim. Biophys. Acta 290:22

    PubMed  Google Scholar 

  20. Hubbell, W.L., McConnell, H.M. 1971. Molecular motion in spin-labeled phospholipids and membranes.J. Am. Chem. Soc. 93:314

    PubMed  Google Scholar 

  21. Ito, T., Ohnishi, S., Ishinaga, M., Kito, M. 1975. Synthesis of a new phosphatidylserine spin-label and calcium-induced lateral phase separation in phosphatidylserine-phosphatidylcholine membranes.Biochemistry 14:3064

    PubMed  Google Scholar 

  22. Jost, P.C., Libertini, L.J., Herbert, V.C., Griffith, O.H. 1971. Lipid spin labels in lecithin multilayers: A study of motion along fatty acid chains.J. Mol. Biol. 59:77

    Article  PubMed  Google Scholar 

  23. Kirkpatrick, F.H., Sandberg, H.E. 1973. Effects of preparation method on reversible conformational changes induced by neutral salts in spin-labeled erythrocyte membranes.Arch. Biochem. Biophys. 156:653

    Article  PubMed  Google Scholar 

  24. Kirkpatrick, F.H., Sandberg, H.E. 1973. Effects of anionic surfactants, nonionic surfactants, and neutral salts, on the conformation of spin-labeled erythrocyte membrane proteins.Biochim. Biophys. Acta 298:209

    PubMed  Google Scholar 

  25. Kuhn, E., Dorow, W., Kahlke, W., Pfisterer, H. 1968. Myotonie nach 20,25 diazacholesterin bei der Ratte. Elektromyographie, Mechanokardiographie, Fettsäurenanalyse der Phosphatide des Skeletmuskels.Klin. Wschr. 46:1043

    Article  PubMed  Google Scholar 

  26. Lipicky, R.J., Bryant, S.H. 1972. Temperature effects on cable parameters and K efflux in normal and myotonic goat muscle.Am. J. Physiol. 222:213

    PubMed  Google Scholar 

  27. Lipicky, R.J., Bryant, S.H. 1973. A biophysical study of the human myotonias.In: New Developments in Electromyography and Clinical Neurophysiology. J.E. Desmedt, editor. Vol. 1, p. 451. Karger Press, Basel

    Google Scholar 

  28. Lowry, O.H., Rosebrough, N.J., Farr, A.C., Randall, R.J. 1951. Protein measurement with the Folin phenol reagent.J. Biol. Chem. 193:265

    PubMed  Google Scholar 

  29. Mason, R.P., Polnaszek, C.F., Freed, J.H. 1974. Comments on the interpretation of electron spin resonance spectra of spin labels undergoing very anisotropic rotational reorientation.J. Phys. Chem. 78:1324

    Article  Google Scholar 

  30. Munsat, T.L. 1967. Therapy of myotonia.Neurology 17:359

    PubMed  Google Scholar 

  31. Peter, J.B., Andiman, R.M., Bowman, R.L., Nagatomo, T. 1973. Myotonia induced by diazacholesterol: Increased (Na++K+)-ATPase activity of erythrocyte ghosts and development of cataracts.Exp. Neurol. 41:738

    Article  PubMed  Google Scholar 

  32. Peter, J.B., Fiehn, W. 1973. Myotonia induced by diazacholesterol accumulation of desmosterol and increased ATPase activity of rat sarcolemma.Science 173:910

    Google Scholar 

  33. Peter, J.B., Stempel, K.E., Dromgoole, S.H., Campion, D.S., Bowman, R.L., Nagatomo, T., Andiman, R.M. 1975. The effect of a high cholesterol diet on 20,25-diazacholesterol-induced myotonia.Exp. Neurol. 49:429

    Article  PubMed  Google Scholar 

  34. Roses, A.D., Butterfield, D.A., Appel, S.H., Chesnut, D.B. 1975. Phenytoin and membrane fluidity in myotonic dystrophy.Arch. Neurol. 32:535

    PubMed  Google Scholar 

  35. Rüdel, R., Senges, J. 1972. Experimental myotonia in mammalian skeletal muscle: Changes in membrane properties.Pfluegers Arch. Gesamte Physiol. 331:324

    Article  Google Scholar 

  36. Rüdel, R., Senges, J. 1973. Experimental myotonia.In: New Developments in Electromyography and Clinical Neurophysiology. J.E. Desmedt, editor. Vol. 1, p. 483. Karger Press, Basel

    Google Scholar 

  37. Sandberg, H.E., Bryant, R.G., Piette, L.H. 1969. Studies on the location of sulfhydryl groups in erythrocyte membranes with magnetic resonance spin probes.Arch. Biochem. Biophys. 133:144

    PubMed  Google Scholar 

  38. Schneider, H., Smith, I.C.P. 1970. A study of the structural integrity of spin-labelled proteins in some fractions of human erythrocyte ghosts.Biochim. Biophys. Acta 219:73

    PubMed  Google Scholar 

  39. Seiler, D., Kuhn, E., Fiehn, W., Hasselbach, W. 1970. The properties of sarcoplasmic vesicles from rats with 20,25-diazacholesterol induced myotonia.Eur. J. Biochem. 12:375

    Article  PubMed  Google Scholar 

  40. Singer, S.J. 1971. The molecular organization of biological membranes.In. Structure and Function of Biological Membranes. L.I. Rothfield, editor. p. 145. Academic Press, New York

    Google Scholar 

  41. Somers, J.E., Winer, N. 1966. Reversible myopathy and myotonia following administration of a hypercholesterolemic agent.Neurology 16:761

    Google Scholar 

  42. Steinberg, S., Botelho, S. 1962. Myotonia in a horse.Science 137:979

    PubMed  Google Scholar 

  43. Winer, N., Klachko, D.M., Burns, T.W., Baer, R.D. 1965. Electromyographic myotonic responses induced by cholesterol-lowering agents.Arch. Phys. Med. 46:499

    Google Scholar 

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  1. Department of Chemistry, University of Kentucky, 40506, Lexington, Kentucky

    D. Allan Butterfield & Wesley E. Watson

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  1. D. Allan Butterfield
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  2. Wesley E. Watson
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Allan Butterfield, D., Watson, W.E. Electron spin resonance studies of an animal model of human congenital myotonia: Increased erythrocyte membrane fluidity in rats with 20,25-diazacholesterol-induced myotonia. J. Membrain Biol. 32, 165–176 (1977). https://doi.org/10.1007/BF01905215

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  • DOI: https://doi.org/10.1007/BF01905215

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