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The dysmyelinating mouse mutations shiverer (shi) and myelin deficient (shi mld)

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Abstract

Shiverer (shi/shi) is an autosomal recessive mouse mutation that produces a shivering phenotype in affected mice. A shivering gait can be seen from a few weeks after birth until their early death, which occurs between 50 and 100 days. The central nervous system of the mutant mouse is hypomyelinated but the peripheral nervous system appears normal. The myelin of the CNS, wherever present, is not well compacted and lacks the major dense line. Myelin basic protein (MBP), which is associated with the major dense line, is absent, and this is due to a deletion of the major part of the gene encoding MBP. Transgenic shiverer mice that have integrated and express the wild-type mouse MBP transgene no longer shiver and have normal life spans. Conversely, normal mice that have integrated an antisense MBP transgene, shiver. Myelin deficient shimld/shimld is allelic to shiverer (shi/shi) but the mutant mouse is less severely affected. Although MBP is present in the CNS, it is low in quantity and is not developmentally regulated. The gene encoding MBP has been both duplicated and inverted. Transgenic shimld/shimld mice with the wild-type MBP transgene have normal phenotypes.

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References

  • Abbott, C., Povey, S., Vivian, N., and Lovell-Badge, R. (1988). PCR as a rapid screening method for transgenic mice.TIGs 44:325.

    Google Scholar 

  • Akowitz, A. A., Barbarese, E., Scheld, K., and Carson, J. H. (1987). Structure and expression of myelin basic protein gene sequences in mild mutant mouse: Reiteration and rearrangement of the MBP gene.Genetics 116:447–464.

    Google Scholar 

  • Arquint, M., Roder, J., Chia, L.-S., Down, J., Wilkinson, D., Bayley, H., Braun, P., and Dunn, R. (1987). Molecular cloning and primary structure of myelin-associated glycoprotein.Proc. Natl. Acad. Sci. USA 84:600–604.

    Google Scholar 

  • Barbarese, E., Braun, P. E., and Carson, J. H. (1977). Identification of pre-large and pre-small basic proteins in mouse myelin and their structural relationship to large and small basic proteins.Proc. Natl. Acad. Sci. USA 74:3360–3364.

    Google Scholar 

  • Barbarese, E., Carson, J. H., and Braun, P. E. (1978).J. Neurochem. 31:779–782.

    Google Scholar 

  • Barbarese, E., Nielson, M. L., and Carson, J. H. (1983). The effect of the shiverer mutation on myelin basic protein expression in homozygous and heterozygous mouse brain.J. Neurochem. 40:1680–1686.

    Google Scholar 

  • Benjamin, J. A., and Smith, M. E. (1984). Metabolism of myelin. In Morrell, P. (ed.),Myelin, Plenum Press, New York, pp. 225–258.

    Google Scholar 

  • Benjamin, J. A., Iwata, R., and Hazlett, J. (1978). Kinetics of entry of proteins into the myelin membrane.J. Neurochem. 31:1077.

    Google Scholar 

  • Bernier, L., Alvarez, F., Norgard, G. M., Raible, D. W., Mentaberry, A., Schembri, J. G., Sabatini, D. D., and Colman, D. R. (1987). Molecular cloning of a 2′,3′-cyclic nucleotide 3′-phosphodiesterase: mRNAs with different 5′ ends encode the same set of proteins in nervous and lymphoid tissues.J. Neurosci. 7:2703–2710.

    Google Scholar 

  • Biddle, F., March, E., and Miller, J. R. (1973). Research news.Mouse News Lett. 48: 24.

    Google Scholar 

  • Billings-Gagliardi, S., and Wolf, M. K. (1988). Shiverer/jimpy double mutant mice. IV. Five combinations of allelic mutations produce three morphological phenotypes.Brain Res. 455:271–282.

    Google Scholar 

  • Billings-Gagliardi, S., Wolf, M. K., Kirschner, D. A., and Kerner, A.-L. (1986). Shiverer and jimpy double mutant mice. II. Morphological evidence supports reciprocal intergenic suppression.

  • Billings-Gagliardi, S., Kerner, A.-L., Kirschner, D. A., and Wolf, M. K. (1987). Shiverer and jimpy double mutant mice. III. Comparison of shimld and jpmld and shi and jp phenotypes demonstrates dissimilar interactions of allelic mutations.Mol. Brain Res. 2:199–214.

    Google Scholar 

  • Bird, T. D., Farrell, D. F., and Sumo, S. M. (1978). Brain lipid composition of the shiverer mouse: Genetic defect in myelin development.J. Neurochem. 31:387–391.

    Google Scholar 

  • Bourre, J. M., Jacque, C., Delasalle, A., Nguyen-Legros, J., Dumont, O., Lachapelle, F., Raoul, M., Alvarez, C., and Baumann, N. (1980). Density profile and basic protein measurements in the myelin range of particulate material from normal developing mouse brain and from neurological mutants (jimpy, quaking, trembler, shiverer and its mild allele) obtained by zonal centrifugation.J. Neurochem. 35:458–464.

    Google Scholar 

  • Brinster, R. L., Chen, H. Y., Trumbauer, M. E., Yaghe, M. K., and Palmiter, R. D. (1985). Factors affecting the efficiency of introducing foreign DNA into mice by microinjecting eggs.Proc. Natl. Acad. Sci. USA 82:4438–4442.

    Google Scholar 

  • Brinster, R. L., Allen, J. M., Bohringer, R. R., Gelinas, R. E., and Palmiter, R. D. (1988). Introns increase transcriptional efficiency in transgenic mice.Proc. Natl. Acad. Sci. USA 85:836–840.

    Google Scholar 

  • Cammer, W., and Tansey, F. A. (1988). Localization of glial cell antigens in the brains of young normal mice and the dysmyelinating mutant mice, jimpy and shiverer.J. Neurosci. Res. 20:23–31.

    Google Scholar 

  • Campagnoni, A. T. (1988). Molecular biology of myelin proteins from the central nervous system.J. Neurochem. 51:1–14.

    Google Scholar 

  • Carnegie, P. R. (1971). Amino acid sequence of the encephalitogenic basic protein from human myelin.Biochem. J. 123:57–67.

    Google Scholar 

  • Carson, J. H., Nelson, M. L., and Barbarese, E. (1983). Developmental regulation of myelin basic protein expression in mouse brain.Dev. Biol. 96:485–492.

    Google Scholar 

  • Chernoff, G. F. (1981). Shiverer: An autosomal recessive mutant mouse with myelin deficiency.J. Hered. 72:128.

    Google Scholar 

  • Colman, D. R., Kreibich, G., Frey, A. B., and Sabatini, D. D. (1982). Synthesis and incorporation of myelin polypeptides in CNS myelin.J. Cell Biol. 95:598–608.

    Google Scholar 

  • Costantini, F., and Lacy, E. (1981). Introduction of rabbit B-globin into the mouse germ line.Nature 294:92–94.

    Google Scholar 

  • Costantini, F., Chada, K., and Magram, J. (1986). Correction of murine B-thalassemia by gene transfer into the germ line.Science 233:1192–1194.

    Google Scholar 

  • Cowan, J. (1980).Mouse News Lett. 63:14.

    Google Scholar 

  • Dautigny, A., Alliee, P. M., d'Auriol, L., Pham Dinh, D., and Nussbaum, J.-L. (1985). Molecular cloning and nucleotide sequence of a cDNA coding for rat brain myelin proteolipid.FEBS Lett. 188:33–36.

    Google Scholar 

  • DeFerra, F., Engh, H., Hudson, L., Kamholz, J., Puckett, C., Molineaux S., and Lazzarini, R. A. (1985). Alternative splicing acounts for the four forms of myelin basic protein.Cell 43: 721–727.

    Google Scholar 

  • Diehl, H.-J., Schaich, M., Budzinski, R.-M., and Stoffel, W. (1986). Individual exons encode the integral membrane domains of myelin proteolipid protein.Proc. Natl. Acad. Sci. USA 83:9807–9811.

    Google Scholar 

  • Doolittle, D. P., and Schweikart, K. M. (1977). Myelin deficient, a new neurological mutant in the mouse.J. Hered. 68:331–332.

    Google Scholar 

  • Doolittle, D. P., Baumann, N., and Chernoff, G. (1981). Allelism of two myelin deficiency mutants in the mouse.J. Hered. 72:285.

    Google Scholar 

  • Drummond, R. J., and Dean, G. (1980). Comparison of 2′,3′ cyclic nucleotide 3′-phosphodiesterase and the major component of Wolfgram protein W1.J. Neurochem. 35:1155–1165.

    Google Scholar 

  • Dunkley, P. R., and Carnegie, P. R. (1974). Amino acid sequence of the smaller basic protein from rat myelin.Biochem. J. 141:243–255.

    Google Scholar 

  • Dupouey, P., Jacque, C., Bourre, J. M., Cesselin, F., Privat, A., and Baumann, N. (1979). Immunochemical studies of myelin basic protein in shiverer mouse devoid of major dense line of myelin.Neurosci. Lett. 12:113–118.

    Google Scholar 

  • Eylar, E. H., Brostoff, S., Hashim, G., Caccam, J., and Burnett, P. (1971). Basic A1 protein of myelin membrane.J. Biol. Chem. 246:5770–5784.

    Google Scholar 

  • Ganser, A. L., Kerner A.-L., Brown, B. J., Davisson M. T., and Kirschner, D. A. (1988a). A survey of neurological mutant mice. I. Lipid composition of myelinated tissue in known myelin mutants.Dev. Neurosci. 10:99–122.

    Google Scholar 

  • Ganser, A. L., Kerner, A.-L., Brown, B. J., Davisson, M. T., and Kirschner, D. A. (1988b). A survey of neurological mutant mice. II. Lipid composition of myelinated tissue in possible myelin mutants.Dev. Neurosci. 10:123–140.

    Google Scholar 

  • Gordon, J. W., Scangos, G. A., Plotkin, D. J. Barbosa, J. A., and Ruddle, F. H. (1980). Genetic transformation of mouse embryos by microinjection of purified DNA.Proc. Natl. Acad. Sci. USA 77:7380–7384.

    Google Scholar 

  • Gould, R. M. (1985). In Wiggins, R. C. (ed.),Developmental Neurochemistry, McCandless and Enna, pp. 47–99.

  • Griffiths, S. A., and Blair, G. E. (1988). Immunochemical analysis of myelin proteins of the rat central nervous system.Biochem. Soc. Trans. 16:614–615.

    Google Scholar 

  • Grosveld, F., van Assendelft, G. B., Greaves, D. R., and Kollias, G. (1987). Position-independent high-level expression of the human β-globin gene in transgenic mice.Cell 51:975–985.

    Google Scholar 

  • Hirano, A., Sax, D. S., and Zimmerman, H. M. (1969). The fine structure of the cerebella of jimpy mice and their “normal” littermates.J. Neuropathol. Exp. Neurol. 28:388–400.

    Google Scholar 

  • Hogan, B. L. M., Costantini, F., and Lacy, E. (1986).Manipulation of the Mouse Embryo: A Laboratory Manual. Cold Spring Harbor Laboratory, Cold Spring Harbor, N.Y.

    Google Scholar 

  • Holmes, E., Hermanson, G., Cole, R., and DeVellis, J. (1988). Developmental expression of glialspecific mRNAs in primary cultures of rat brain visualized by in situ hybridization.J. Neurosci. Res. 19:389–396.

    Google Scholar 

  • Hughes, S. M. (1988). Ciliary neurotrophic factor (CNTF) induces type-2 astrocyte differentiation in culture.Nature 335:70–73.

    Google Scholar 

  • Huxley, A. F., and Stampfli, R. (1949). Evidence for saltatory conduction in peripheral myelinated nerve fibers.J. Physiol. (London) 108:315

    Google Scholar 

  • Ishaque, A., Roomi, M. W., Szymanska, I., Kowalski, S., and Eylar, E. H. (1980). The Po glycoprotein of peripheral nerve myelin.Can. J. Biochem. 58:913–921.

    Google Scholar 

  • Jacque, C., Delasalle, A., Raoul, M., and Baumann, N. (1983). Myelin basic protein deposition in the optic and sciatic nerves of dysmyelinating mutants quaking, jimpy, trembler, mld and shiverer during development.J. Neurochem. 41:1335–1340.

    Google Scholar 

  • Jaenish, R. (1988). Transgenic animals.Science 240:1468–1474.

    Google Scholar 

  • Jirounek, P., Vitus, J., Pralong, W. F., and Straub, R. W. (1988). Calcium efflux and intracellular exchangeable calcium in mammalian nonmyelinated nerve fibers.J. Membr. Biol. 103:121–134.

    Google Scholar 

  • Karthigasan, J., and Kirschner, D. A. (1988). Membrane interactions are altered in myelin isolated from central and peripheral nervous system tissues.J. Neurochem. 51:228–236.

    Google Scholar 

  • Katsuki, M., Sato, M., Kimura, M., Yokoyama, M., Kobayashi, K., and Nomura, T. (1988). Conversion of normal behavior to shiverer by myelin basic protein antisense cDNA in transgenic mice.Science 241:593–595.

    Google Scholar 

  • Kerner, A.-L., and Carson, J. H. (1983). The expression of myelin proteins in heterozygous and hemizygous jimpy mice.Trans. Am. Soc. Neurochem. 14:128.

    Google Scholar 

  • Kerner, A.-L., and Carson, J. H. (1986). Shiverer and jimpy double mutant mice. I. Biochemical evidence for reciprocal intergenic suppression.Brain Res. 374:45–53.

    Google Scholar 

  • Kirschner, D. A., and Ganser, A. L. (1980). Compact myelin exists in the absence of basic protein in the shiverer mutant mouse.Nature (London) 283:207–210.

    Google Scholar 

  • Knapp, P. E., Skoff, R. P., and Redstone, D. W. (1986). Oligodendroglial death in jimpy mice: An explanation for the myelin deficient.J. Neurosci. 6:2813–2822.

    Google Scholar 

  • Kuchler, S., Fressinaud, C., Sarlieve, L. L., Vincendon, G., and Zanetta, J.-P. (1988). Cerebellar soluble lectin is responsible for cell adhesion and participates in myelin compaction in cultured rat oligodendrocytes.Dev. Neurosci. 10:199–212.

    Google Scholar 

  • LaChapelle, F., deBancque, C., Jacque, C., Bourre, J. M., Delasalle, A., Doolittle, D., Hauw, J. J., and Baumann, N. (1981). Comparison of morphological and biochemical defects of two probably allelic mutations of the mouse, myelin deficient (mild) and shiverer (shi). InProceedings: International Colloquium on Neurological Mutations Affecting Myelinisation, Seillac, France, April 1980, Elsevier North-Holland, Amsterdam.

    Google Scholar 

  • Lai, C., Brow, M. A., Nave, K. A., Noronha, A. B., Quarles, R., Bloom, F. E., Milner, R. J., and Sutcliffe, J. G. (1987). Two forms of 1B236/myelin-associated glycoprotein (MAG), a cell adhesion molecule for postnatal neural development, are produced by alternative splicing.Proc. Natl. Acad. Sci. USA 84:4337–4341.

    Google Scholar 

  • Lai, E., Wilson, R., and Hood, L. (1989). Physical maps of the mouse and human immunoglobulin like loci.Adv. Immunol. 46 (in press).

  • Lees, M. B., and Brostoff, S. W. (1984). Proteins in myelin. In Morell, P. (ed.),Myelin, 2nd ed., Plenum Press, New York and London, pp. 197–224.

    Google Scholar 

  • Lemke, G. (1987). Molecular biology of the genes encoding the major myelin proteins. In Heineman, S., and Patrick, J. (eds.),Molecular Neurobiology.

  • Lemke, G., and Axel, R. (1985). Isolation and sequence of a cDNA encoding the major structural protein of peripheral myelin.Cell 40:501–508.

    Google Scholar 

  • Lemke, G., Lamar, E., and Patterson, J. (1988). Isolation and analysis of the gene encoding peripheral myelin protein zero.Neuron 1:73–83.

    Google Scholar 

  • Macklin, W. B., Gardinier, M. V., King, K. D., and Kampf K. (1987). An AG→GG transition at a splice site in the myelin proteolipid protein gene in jimpy mice results in the removal of an exon.FEBS Lett. 223:417–421.

    Google Scholar 

  • Martenson, R. F., Deibler, G. E., and Kiss, M. W. (1971). The occurrence of two myelin basic proteins in the central nervous system of rodents in the suborders Myomorpha and Scieromorpha.J. Neurochem. 18:2427–2433.

    Google Scholar 

  • Martenson, R. E., Diebler, G. E., Kies, M. W., McNeally, S. S., Shapira, R., and Kibler, R. F. (1972). Differences between the two myelin basic proteins of the rat central nervous system.Biochim. Biophys. Acta 263:193–203.

    Google Scholar 

  • Matthieu, J. M., Omlin, F. X., Ginalski-Winkelmann, H., and Cooper, B. J. (1984). Myelination in the CNS of mld mutant mice: Comparison between composition and structure.Dev. Brain Res. 13:149–158.

    Google Scholar 

  • Meier, C., and Bischoff, A. (1975). Oligodendroglial cell development in jimpy mice and controls. An electron microscopic study in the optic nerve.J. Neurol. Sci. 26:517–528.

    Google Scholar 

  • Mikoshiba, K., Kohsaka, S., Takamatsu, K., and Tsukada, Y. (1981). Neurochemical and morphological studies on the myelin of peripheral nervous system from shiverer mutant mice: Absence of basic proteins common to central nervous system.Brain Res. 204:455–460.

    Google Scholar 

  • Milner, R. J., and Sutcliffe, J. G. (1983). Gene expression in rat brain.Nucl. Acids Res. 11: 5497–5520.

    Google Scholar 

  • Milner, R. J., Lai, C., Nave, K.-A., Lenoir, D., Ogata, J., and Sutcliffe, J. G. (1985). Nucleotide sequences of two mRNAs for rat brain myelin proteolipid protein.Cell 42: 931–939.

    Google Scholar 

  • Molineaux, S. M., Engh, H., DeFerra, F., Hudson, L., and Lazzarini, R. A. (1986). Recombination within the myelin basic protein gene created the dysmyelinating shiverer mouse mutation.Proc. Natl. Acad. Sci. USA 83:7542–7546.

    Google Scholar 

  • Nave, K.-A., Lai, C., Bloom, F. E., and Milner, R. J. (1986). Jimpy mutant mouse: A 74 base deletion in the mRNA for myelin proteolipid protein and evidence for a primary defect in RNA splicing.Proc. Natl. Acad. Sci. USA 83:9264–9268.

    Google Scholar 

  • Nave, K.-A., Lai, C., Bloom, F. E., and Milner, R. J. (1987). Splice site selection in the proteolipid protein (PLP) gene transcript and primary structure of the DM-20 protein of CNS myelin.Proc. Natl. Acad. Sci. USA 84:5665–5669.

    Google Scholar 

  • Newman, S., Kitamura, K., and Campagnoni, A. T. (1987). Identification of a cDNA clone coding for a fifth form of myelin basic protein in the mouse.Proc. Natl. Acad. Sci. USA 84:886–890.

    Google Scholar 

  • Noebels J. L. (1986). Mutational analysis of inherited epilepsies.Adv. Neurol. 21:749–757.

    Google Scholar 

  • Norton, W. T., and Poduslo, S. E. (1973). Myelination in the rat brain: Method of myelin isolation.J. Neurochem. 21:759.

    Google Scholar 

  • Okano, H., Miura, M., Moriguchi, A., Ikenaka, K., Tsukada, Y., and Mikoshiba, K. (1987). Inefficient transcription of the myelin basic protein gene possibly causes hypomyelination in myelin-deficient mutant mice.J. Neurochem. 48:470–476.

    Google Scholar 

  • Okano, H., Tamura, T., Miura, M., Aoyama, A., Ikenaka, K., Oshimura, M., and Mikoshiba, K. (1988a). Gene organization and transcription of duplicated MBP genes of myelin deficient (shi mld) mutant mouse.EMBO J. 7:77–83.

    Google Scholar 

  • Okano, H., Ikenaka, K., and Mikoshiba, K. (1988b). Recombination within the upstream gene of duplicated myelin basic protein genes of myelin deficient (mld) mouse results in the production of antisense RNA.EMBO J. 7:3407–3412.

    Google Scholar 

  • Palmiter, R.D., and Brinster, R. L. (1985). Transgenic mice.Cell 41:343–345.

    Google Scholar 

  • Popko, B., Puckett, C., Lai, E., Shine, H. D., Readhead, C., Takahashi, N., Hunt, S., Sidman, R. L., and Hood, L. (1987). Myelin deficient mice: Expression of myelin basic protein and generation of mice with varying levels of myelin.Cell 48:713–721.

    Google Scholar 

  • Popko, B., Puckett C., and Hood, L. (1988). A novel mutation in myelin-deficient mice results in unstable myelin basic protein gene transcripts.Neuron 1:221–225.

    Google Scholar 

  • Popko, B., Readhead, C., Dausman, J., and Hood, L. E. (1989). Production of transgenic mice.Meth. Enzymol. (in press).

  • Privat, A., Robain, O., and Mandel, P. (1972). Aspects ultrastructuraux due corps calleaux chez la souris jimpy.Acta Neuropath. (Berlin) 21.

  • Privat, A., Jacque, C., Bourre, J. M., Dupouey, P., and Baumann, N. (1979). Absence of major dense line in the myelin of the mutant mouse shiverer.Neurosci. Lett. 12:107–112.

    Google Scholar 

  • Puckett, C., Hudson, L., Ono, K., Friedrich, V., Benecke, J., Dubois-Dalcq, M. and Lazzarini, R. A. (1987). Myelin-specific proteolipid protein is expressed in myelinating Schwann cells but is not incorporated into myelin sheaths.J. Neurosci. Res. 18:511–518.

    Google Scholar 

  • Quarles, R. H. (1980). Glycoproteins from central and peripheral myelin. In Hashim, G. (ed.),Myelin: Chemistry and Biology, Alan R. Liss, New York, pp. 55–77.

    Google Scholar 

  • Raff, M. C. (1988). Astrocyte-derived PDGF observes the clock that times oligodendrocyte development in culture.Nature 333:562–565.

    Google Scholar 

  • Raff, M. C., Miller, R. H., and Noble, M. (1983). A glial progenitor cell that developsin vitro an astrocyte or an oligodendrocyte depending on culture medium.Nature 303:390–396.

    Google Scholar 

  • Raine, C. S. (1984). Morphology of myelin and myelination. In Morell, P. (ed.),Myelin, Plenum Press, New York, pp. 1–41.

    Google Scholar 

  • Readhead, C., Popko, B., Takahashi, N., Shine, H. D., Saavedra, R. A., Sidman, R. L., and Hood, L. (1987). Expression of a myelin basic protein gene in transgenic shiverer mice: Correction of the dysmyelinating phenotype.Cell 48:703–712.

    Google Scholar 

  • Ritchie, J. M. (1984) Physiological basis of conduction in myelinated nerve fibers. In Morell, P. (ed.),Myelin, 2nd ed., Plenum Press, New York and London, pp. 117–145.

    Google Scholar 

  • Roach, A., Boylan, K., Horvath, S., Prusiner, S. B., and Hood, L. (1983). Characterization of cloned cDNA representing rat myelin basic protein: absence of expression in brain of shiverer mutant mice.Cell 34:799–806.

    Google Scholar 

  • Roach, A., Takahashi, N., Pravtcheva, D., Ruddle, F., and Hood, L. (1985). Chromosomal mapping of mouse myelin basic protein gene and structure and transcription of the partially deleted gene in shiverer mutant mice.Cell 42:149–155.

    Google Scholar 

  • Roch, J., Brown-Luedi, M., Cooper, B. J., and Matthieu, J. (1986). Mice heterozygous for the mld mutation have intermediate levels of myelin basic protein mRNA and its translation products.Mol. Brain Res. 1:137–144.

    Google Scholar 

  • Rosenbluth, J. (1980a). Central myelin in the mouse mutant shiverer.J. Comp. Neurol. 194:639–648.

    Google Scholar 

  • Rosenbluth, J. (1980b). Peripheral myelin in the mouse mutant shiverer.J. Comp. Neurol. 193:729–739.

    Google Scholar 

  • Rosenbluth, J. (1981). Axoglial junctions in the mouse mutant shiverer.Brain Res. 208:283–297.

    Google Scholar 

  • Rosenbluth, J. (1987). Abnormal axoglial junction in the myelin-deficient rat mutant.J.Neurocytol. 16:497–509.

    Google Scholar 

  • Rosenbluth, J. (1988). Role of glial cells in the differentiation and function of myelinated axons.Int. J. Dev. Neurosci. 6:3–24.

    Google Scholar 

  • Rosenbluth, J., and Hasegawa, M. (1988). Spinal cord injury or spinal anesthesia eliminates seizures in myelin-deficient rats.Neurosci. Lett. 84:68–72.

    Google Scholar 

  • Rosenfeld, M. G., Crenshaw, E. B., III, Lira, S. A., Swanson, L., Borrelli, E., Heyman, R., and Evans, R. M. (1988). Transgenic mice: Applications in the study of the nervous system.Annu. Rev. Neurosci. 11:353–372.

    Google Scholar 

  • Saavedra, R. A., Fors, L., Aebersold, R. H., Arden, B., Horvath, S., Sanders, J., and Hood, L. (1989). The myelin proteins of the shark brain are similar to the myelin proteins of the mammalian peripheral nervous system.J. Mol. Evol. (in press).

  • Salzer, J. L., Holmes, W. P., and Colman, D. R. (1987). The amino acid sequences of the myelin-associated glycoproteins: Homology to the immunoglobulin gene superfamily.J. Cell Biol. 335:70–73.

    Google Scholar 

  • Shen, X.-Y., Billings-Gagliardi, S., Sidman, R. L., and Wolf, M. K. (1985). Myelin deficient (shimld) mutant allele: Morphological comparison with shiverer (shi) allele on a B6C3 mouse stock.Brain Res. 360:235–247.

    Google Scholar 

  • Shine, H. D., Readhead, C., Popko, B., Hood, L., and Sidman, R. L. (1988). Morphometric analysis of myelin in the optic nerve of transgenic shiverer mice.Soc. Neurosci. Abstr. 14:623.

    Google Scholar 

  • Sidman, R. J., Dickie, M. M., and Appel, S. H. (1964). Mutant mice (quaking and jimpy) with deficient myelin in the central nervous system.Science 144:309–311.

    Google Scholar 

  • Sidman, R. J., Conover, C. S., and Carson, J. H. (1985). Shiverer gene maps near the distal end of chromosome 18 in the house mouse.Cytogenet. Cell. Genet. 39:241–245.

    Google Scholar 

  • Sims, T. J., Gilmore, S. A., and Waxman, S. G. (1988). Temporary adhesions between axons and myelin-forming processes.Dev. Brain Res. 40:223–232.

    Google Scholar 

  • Singh, H., and Spritz, N. (1974). Polypeptide components of myelin from rat peripheral nerve.Biochim. Biophys. Acta. 351:379–386.

    Google Scholar 

  • Sprinkle, T. J., Wells, M. R., Garver, F. A., and Smith, D. B. (1980). Studies on the Wolfgram high molecular weight CNS myelin protein: Relationship to 2′,3′-cyclic nucleotide-3′-phosphodiesterase.J. Neurochem. 35:1200–1208.

    Google Scholar 

  • Stoffel, W., Hillein, H., and Giersifen (1984).Proc. Natl. Acad. Sci. USA 81:5012–5016.

    Google Scholar 

  • Stoffyn, P., and Folch-Pi, J. (1971).Biochem. Biophys. Res. Commun. 44:157–161.

    Google Scholar 

  • Sutcliffe, J. G. (1988a). The genes for myelin revisited.Trends Genet. 4:211–213.

    Google Scholar 

  • Sutcliffe, J. G. (1988b). mRNA in the mammalian nervous system.Annu. Rev. Neurosci. 11:157–198.

    Google Scholar 

  • Takahashi, N., Roach, A., Teplow, D. B., Prusiner, S. B., and Hood, L. (1985). Cloning and characterization of the myelin basic protein gene from mouse: One gene can encode both 14 kb and 18.5 kb MBPs by the alternate use of exons.Cell 42:139–148.

    Google Scholar 

  • Trapp, B. D., Moench, T., Pulley, M., Barbosa, E., Tennekoon, G., and Griffin, J. (1987). Spatialsegregation of mRNA encoding myelin-specific proteins.Proc. Natl. Acad. Sci. USA 84:7773–7777.

    Google Scholar 

  • Trapp, B. D., Bernier, L., Andrews, S. B., and Colman, D. R. (1988a). Cellular and subcellular distribution of 2′,3′-cyclic nucleotide 3′-phosphodiesterase and its mRNA in the rat central nervous system.J. Neurochem. 51:859–868.

    Google Scholar 

  • Trapp, B. D., Hauer, P., and Lemke, G. (1988b). Axonal regulation of myelin protein mRNA levels in actively myelinating Schwann cells.J. Neurosci. 8:3515–3521.

    Google Scholar 

  • Verity, A. N., and Capagnoni, A. T. (1988) Regional expression of myelin proteins genes in the developing mouse brain:In situ hybridization studies.J Neurosci. Res. 21:238–248.

    Google Scholar 

  • Vogel, U. S., and Thompson, R. J. (1988). Molecular structure, localization, and possible functions of the myelin-associated enzyme 2′,3′-cyclic nucleotide 3′-phosphodiesterase.J. Neurochem. 50:1667–1677.

    Google Scholar 

  • Waehneldt, T. V., Matthieu, J.-M., and Jeserich, G. (1986). Appearance of myelin proteins during vertebrate evolution.Neurochem. Int. 9:463–474.

    Google Scholar 

  • Waxman, S. G., and Black, J. A. (1988). Unmyelinated and myelinated axon membrane from rat corpus callosum: Differences in macromolecular structure.Brain Res. 453:337–343.

    Google Scholar 

  • Westphal, H. (1989). Transgenic animals and biotechnology.FASEB J. 117–120.

  • Wiggins, R. C., Benjamins, J. A., and Morell, P. (1975). Appearance of myelin proteins in rat sciatic nerve during development.Brain Res. 89:99–106.

    Google Scholar 

  • Willard, H. F., and Riordan, J. R. (1985). Assignment of the gene for myelin proteolipid protein to the x chromosome: Implications for x-linked myelin disorders.Science 230:940–942.

    Google Scholar 

  • Wolf, M. K., and Billings-Gagliardi, S. (1988). Quaking/shiverer double mutant mice: Morphological phenotypes support possible dual actions of the shiverer locus.Brain Res. 461:257–273.

    Google Scholar 

  • Wolfgram, F. (1986). A new proteolipid fraction of the nervous system: I. Isolation and amino acid analysis.J. Neurochem. 13:461–470.

    Google Scholar 

  • Zeller, N. K., Hunkeler, M. J., Campagnoni, A. T., Sprague, J., and Lazzarini, R. A. (1984). The characterization of mouse myelin basic protein specific messenger RNAs using a myelin basic protein cDNA clone.Proc. Natl. Acad. Sci. USA 81:18–22.

    Google Scholar 

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Readhead, C., Hood, L. The dysmyelinating mouse mutations shiverer (shi) and myelin deficient (shi mld). Behav Genet 20, 213–234 (1990). https://doi.org/10.1007/BF01067791

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