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Expression of myelin-specific proteins during development of normal and hypomyelinatedParalytic tremor mutant rabbits

II. Studies on the purified myelin

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Molecular and Chemical Neuropathology

Abstract

An X-linked, recessive paralytic tremor (pt) mutation is characterized by CNS hypomyelination. In our previous work, we presented developmental studies on the expression of several myelin-specific proteins (PLP/DM-20, MBP, CNP, MAG, and MOG) in the brain homogeates of bothpt mutant and age-matched control Chinchilla rabbits aged 1–120 postnatal days. A moderate reduction in all examined proteins and a striking PLP deficiency were observed in thept mutant rabbits. In the present study, we investigated isolated and purified myelin fractions. A severe (approximately 30% of control values) and approximately constant hypomyelination ofpt, mutant CNS was observed during the entire investigated development (28–120 postnatal days). Although the neurological symptoms gradually regressed and a partial recovery of the affected animals usually occurred; no tendency toward regression of the hypomyelination was noticed. Whereas the content of CNP, MBP, and MAG in isolated myelin membrane fractions seemed close to normal, a drastic PLP deficiency was observed. A significantly elevated, amount of MOG was found in the myelin ofpt mutant rabbits. The controversy between the high degree of hypomyelination and the slight reduction in myelin protein marker expression (except for PLP) in mature brain homogenates is discussed with respect to retarded oligodendrocyte maturation and deficient processing of myelin membranes inpt mutant rabbits.

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References

  • Amiguet P., Gardinier M. V., Zanetta J.-M., and Matthieu J.-M. (1992) Purification and partial structural and functional characterization of mouse myelin/oligodendrocyte glycoprotein.J. Neurochem. 58, 1676–1682.

    Article  PubMed  CAS  Google Scholar 

  • Brady R. O. and Quarles R. H. (1988) Developmental and pathophysiological aspects of the myelin associated glycoprotein.Cell. Mol. Neurobiol. 8, 139–148.

    Article  PubMed  CAS  Google Scholar 

  • Burger D., Perruisseau G., Simon M., and Steck A. J. (1992) Comparison of the N-linked oligosaccharide structures of the two major human myelin glycoproteins MAG and Po:Assessment and relative occurrence of oligosaccharide structure by serial lectin affinity chromatography of14C-glycolipids.J. Neurochem. 58, 845–853.

    Article  PubMed  CAS  Google Scholar 

  • Burger D., Steck A. J., Bernard C. A., and Kerlero de Rosbo N. (1993) Human myelin oligodendrocyte glycoprotein: a new member of the L2/HNH-1 family.J. Neurochem. 61, 1822–1827.

    Article  PubMed  CAS  Google Scholar 

  • Campagnoni A. T. and Macklin W. B. (1988) Cellular and molecular aspects of myelin protein gene expression.Mol. Neurobiol. 2, 41–89.

    Article  PubMed  CAS  Google Scholar 

  • Domanska-Janik K., Wikiel H., and Strosznajder J. (1986) Brain lipids of myelin deficient rabbit during development.Neurochem. Pathol. 4, 135–145.

    Article  PubMed  CAS  Google Scholar 

  • Domańska-Janik K., Gajkowska B., de Nechaud B., and Bourre J. M. (1988) Myelin composition and activities of CNPase and Na+, K+-ATPase in hypomyelinatedpt mutant rabbit.J. Neurochem. 50, 122–130.

    Article  PubMed  Google Scholar 

  • Domańska-Janik K., de Nechaud B., Inomata M., Kawashima S., and Zalewska T. (1992) Calcium-activated neutral protease (CANP) in normal and dysmyelinatingparalytic tremor mutant rabbit myelin.Mol Chem. Neuropathol. 16, 273–288.

    Article  PubMed  Google Scholar 

  • Gardinier M. V., Amiguet P., Linington C., and Matthieu J.-M. (1992) Myelin/oligodendrocyte glycoprotein cDNA analysis reveals a unique member of the immunoglobulin gene superfamily.J. Neurosci. Res. 33, 177–187.

    Article  PubMed  CAS  Google Scholar 

  • Gencic S., Abuelo D., Ambler M., and Hudson L. D. (1989) Pelizaeus-Merzbacher disease: an X-linked neurologic disorder of myelin metabolism with a novel mutation in the gene encoding proteolipid protein.Am. J. Hum. Genet. 45, 435–442.

    PubMed  CAS  Google Scholar 

  • Hartman B. K., Agrawal H. C., Agrawal D., and Kalbach S. (1982) Development and maturation of central nervous system myelin: comparison of immunohistochemical localization of proteolipid protein and basic protein in myelin and oligodendrocytes.Proc. Natl. Acad. Sci. USA 79, 4217–4220.

    Article  PubMed  CAS  Google Scholar 

  • Hudson L. D. (1990) Molecular genetics of X-linked mutants.Ann. N. Y. Acad. Sci. 605, 155–165.

    Article  PubMed  CAS  Google Scholar 

  • Koeppen A. H., Barron K. D., Csiza C. K., and Greenfield E. A. (1988) Comparative immunocytochemistry of Pelizaeus-Merzbacher disease, the jimpy and the myelin-deficient rat.J. Neurol. Sci. 84, 315–327.

    Article  PubMed  CAS  Google Scholar 

  • Laemmli U. K. (1970) Cleavage of structural proteins during the assembly of the head of bacteriophage T4.Nature 227, 680–685.

    Article  PubMed  CAS  Google Scholar 

  • Linington C. and Lassmann H. (1987) Antibody responses in chronic relapsing experimental allergic encephalomyelitis: correlation of serum demyelinating activity with antibody titre to the myelin/oligodendrocyte glycoprotein (MOG).J. Neuroimmunol. 17, 61–69.

    Article  PubMed  CAS  Google Scholar 

  • Lington C., Webb M., and Woodhams P. (1984) A novel myelin-associated glycoprotein defined by a mouse monoclonal antibody.J. Neuroimmunol. 6, 387–396.

    Article  Google Scholar 

  • Lowry O. H., Rosenbrough N. J., Farr A. L., and Randall R. J. (1951) Protein measurement with the Folin phenol reagent.J. Biol. Chem. 193, 265–275.

    PubMed  CAS  Google Scholar 

  • Mitchell L. S., Griffiths I. R., and Brophy P. J. (1990) Rumpshaker mouse. A probable mutation of the PLP gene.Ann. N. Y. Acad. Sci. 605, 394–397.

    Article  Google Scholar 

  • Morell P., Quarles R. H., and Norton W. T. (1989) Formation, structure, and biochemistry of myelin, inBasic Neurochemistry: Molecular, Cellular and Medical Aspects, 4th ed. (Siegel, G. J., Agranoff B. W., Albers R. W., and Molinoff P. B., eds.), pp. 109–136, Raven, New York.

    Google Scholar 

  • Norton W. T. (1984)Oligodendroglia. Plenum Press, New York.

    Google Scholar 

  • Norton W. T. and Poduslo S. E. (1973) Myelination in rat brain: method of myelin isolation.J. Neurochem. 21, 749–757.

    Article  PubMed  CAS  Google Scholar 

  • Nussbaum J. L. and Roussel G. (1983) Immunocytochemical demonstration of the transport of myelin proteolipids through the Golgi apparatus.Cell Tissue Res. 234, 547–559.

    Article  PubMed  CAS  Google Scholar 

  • Osetowska E. and Leszowski F. (1975) Introduction to investigation of hereditary disease of the nervous system onpt rabbit model (in Polish).Neuropatol. Pol. 13, 61–70.

    PubMed  CAS  Google Scholar 

  • Pham-Dinh D., Mattei M.-G., Nussbaum J.-L., Roussel G., Pantarotti P., Roeckel N., Mather I. H., Artzt K., Lindhal K. F., and Dautigny A. (1993) Myelin/oligodendrocyte glycoprotein is a member of the immunoglobulin superfamily encoded within the major histocompatibility complex.Proc. Natl. Acad. Sci. USA 90, 7990–7994.

    Article  PubMed  CAS  Google Scholar 

  • Popot J.-L., Pham-Dinh D., and Dautigny A. (1991) Major myelin proteolipid: the 4-topology.J. Membrane Biol. 120, 233–246.

    Article  CAS  Google Scholar 

  • Quarles R. H. (1990) The biochemistry of myelin in X-linked mutants.Ann. New York Acad. Sci. 605, 135–145.

    Article  CAS  Google Scholar 

  • Quarles R. H., Johnson D., Brady R. O., and Sternberger N. H. (1981) Preparation and characterization of antisera to the myelin-associated glycoprotein.Neurochem. Res. 6, 115–127.

    Article  Google Scholar 

  • Schachner M. (1989) Families of neural adhesion molecules, inCarbohydrate Recognition in Cellular Function. Ciba Foundation Symposium145, pp. 156–168. John Wiley, Chichester.

    Chapter  Google Scholar 

  • Sprinkle T. J. (1989) 2′, 3′-Cyclic nucleotide 3′-phosphodiesterase, an oligoden-drocyte-Schwann cell and myelin-associated enzyme of the nervous system.CRC Crit. Rev. Neurobiol. 4, 235–301.

    CAS  Google Scholar 

  • Sypecka J. and Domańska-Janik K. (1995) Expression of myelin specific proteins during development of normal and hypomyelinatedpt mutant rabbits: studies on the brain homogenates.Mol. Chem. Neuropathol. this issue.

  • Taraszewska A. (1984) Pathology of myelin inpt rabbit (in Polish).Neuropatol. Pol. 22, 205–218.

    PubMed  Google Scholar 

  • Taraszewska A. and Zelman I. (1985) Morphometric studies on myelin development inpt rabbit (in Polish).Neuropatol. Pol. 24, 443–454.

    Google Scholar 

  • Taraszewska A. and Zelman I. (1987) Electron microscopic study of glia inpt rabbits during myelination.Neuropatol. Pol. 25, 351–368.

    PubMed  CAS  Google Scholar 

  • Tosic M., Dolivo M., Amiguet P., Domańska-Janik K., and Matthieu J.-M. (1993). Paralytic tremor (pt) rabbit: a sex-linked mutation affecting proteolipid protein-gene expression.Brain Res. 625, 307–312.

    Article  PubMed  CAS  Google Scholar 

  • Towbin H., Staehelin T., and Gordon J. (1979) Electrophoretic transfer of proteins from polyacrylamide gels to nitrocellulose sheets: procedure and some applications.Proc. Natl. Acad. Sci. USA 76, 4350–4354.

    Article  PubMed  CAS  Google Scholar 

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  1. Department of Neurochemistry, Medical Research Centre, Polish Academy of Sciences, 3 Dworkowa Str., 00-784, Warsaw, Poland

    Joanna Sypecka & Krystyna Domańska-Janik

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  1. Joanna Sypecka
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  2. Krystyna Domańska-Janik
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Sypecka, J., Domańska-Janik, K. Expression of myelin-specific proteins during development of normal and hypomyelinatedParalytic tremor mutant rabbits. Molecular and Chemical Neuropathology 26, 67–78 (1995). https://doi.org/10.1007/BF02814942

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