Myelin pp 489-534 | Cite as

Animal Models of Genetic Disorders of Myelin

  • Edward L. Hogan
  • Seymour Greenfield

Abstract

Improved understanding of the assembly and maintenance of myelin is afforded by the study of animals in which interference with these processes occurs as a result of genetic variation, toxic exposure, immunological attack, viral infection, nutritional or endocrine deficiency, and physical or circulatory insult. Mutant animals are particularly suitable for the biochemical approach, since they make it possible to obtain genetic homogeneity, sufficient quantities for experimental use, and strict control of their nutrition and environment. During the past decade, considerable work has been carried out on several mouse mutants with genetic defects in and limited to the formation of myelin. The first recognized were the jiutpy, quaking, and myelin synthesis deficiency mutants, and study of them contributed importantly to the identification of myelin constituents and clarification of major features of myelin metabolism and myelinogenesis. In recent years, several additional mouse mutants have been described and shown to have myelin disorders with special characteristics that further reveal the biology of myelin. The most notable of these are shiverer, which has an almost complete lack of myelin basic protein; twitcher, which resembles human globoid cell leukodystm ophy in having a severe deficiency of galactosylceramide ß-galactosidase; trembler, which has a primary disorder of peripheral nerve and Schwann cell; and muscular dystrophy, which has amyelinated segments of nerve roots. All these mutants share advantages for experimental study in that the pathology is limited to the myelin, their genetic nature presages likely abnormality of protein structure, serial developmental analysis is possible, and dilfereuees in both phenotype and genotype of myelin disorders have been shown.

Keywords

Sciatic Nerve Schwann Cell Myelin Basic Protein Myelin Protein Organotypic Culture 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Reference

  1. Bird, M. T., Shuttleworth, E., Jr., Koestner, A., and Reinglass, J., 1971, The wobbler mouse mutant: An animal model of hereditary motor system disease, Act. Neuropathol 19: 39.CrossRefGoogle Scholar
  2. Bird, T. D., Farrell, D. F., and Sumi, S. M., 1978, Brain lipid composition of the shiverer mouse: Genetic defect in myelin development, J. Neurochem 31: 387.PubMedCrossRefGoogle Scholar
  3. Bird, ’T. D., Farrell, D. F., Stranahan, S., and Austin, E., 1980, Developmental dissociation of myelin synthesis and “myelin associated” enzyme activities in the shiverer mouse, Neurochem. Res 5: 885.Google Scholar
  4. Biscoe, T. J., Caddy, K. W. T., Fallot, D. J., Pehrson, U. M. M., and Stirling, C. A., 1974, The neurological lesion in the dystrophic mouse, Brain Res. 76: 534.PubMedCrossRefGoogle Scholar
  5. Biscoe, F. J., Caddy, K. W. T., Fallot, D. J., and Pehrsen, V. M. M., 1975, Investigation of cranial and other nerves in the mouse with muscular dystrophy, J. Neurol. Neurosurg. Psychiatry 38: 391.PubMedCrossRefGoogle Scholar
  6. Boegman, J., Aguayo, A. J., and Bray, G. M., 1977, Axoplasmic transport in (Trembler mouse) nerves with a widespread disorder of myelination, Abstr. Am. Assoc. Neuropathol. 53: 590.Google Scholar
  7. Bourque, E. A., Bornstein, M. B., Peterson, E. R., and Suzuki, K., 1983, The twitcher mouse: Myelinogenesis in organotypic culture, Brain Res. 261: 295.PubMedCrossRefGoogle Scholar
  8. Bourre, J. M., and Daudu, O., 1978, Stearyl-alcohol biosynthesis from stearyl-CoA in mouse brain microsomes in normal and dysmyelinating mutants (quaking and jimpy), Neurosci. Lett 7: 225.PubMedCrossRefGoogle Scholar
  9. Bourre, J. M., Follet, S., Daudu, O., and Baumann, N., 1971, Biosynthese des acides gras duns le microsomes de souris normale et quaking, C. R. Acad. Sci. Paris Ser. D 273: 1534.Google Scholar
  10. Bourre, J. M., Cassagne, C., Larrouquere-Regnier, S., and Darriet, D., 1977x, Occurrence of alkane’s in brain myelin: Comparison between normal and quaking mouse, J. Neurchem. 29: 645.CrossRefGoogle Scholar
  11. Bourre, J. M., Paturneau-Jonas, M. Y., Daudu, O. L., and Baumann, N. A., 1977h, Lignoceric acid biosynthesis in the developing brain: Activities of mitochondria] acetyl-CoA-dependent synthesis and microsomel malonyl-CoA chain-elongating system in relation to myelination, Eur. J. Biochem 72: 41.PubMedCrossRefGoogle Scholar
  12. Bourre, J. M., Pollet, S., Daudu, O., LeSaux, F., and Baumann, N., 1977e, Myelin consists of a continuum of particles of different density with varying lipid composition: Major differences arc’ found between normal mice and quaking mutants, Biochimie 59: 819.Google Scholar
  13. 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 mld allele) obtained by zonal centrifugation, J. Neurochem. 35 (2): 458.PubMedCrossRefGoogle Scholar
  14. Bowen, D. M., and Radin, N. S., 1969, Hydrolase activities in brain of neurological mutants Cerebrosidc galactosidase, nitropheny1 galactoside hydro]dse, nitrophenyl glucoside hydrolose and sulphatäse, J. Neurochem 16: 457.PubMedCrossRefGoogle Scholar
  15. Bradley, W. G., and Jaros, E., 1973, Axoplasmic flow in axonal neuropathies. II. Axoplasmic flow in mice with motor neuron disease and muscular dystrophy, Brain 96: 247.PubMedCrossRefGoogle Scholar
  16. Bradley, W. G., and Jenkison, M., 1973, Abnormalities of peripheral nerve in murine muscular dystrophy, J. Neurol. Sci 18: 227.PubMedCrossRefGoogle Scholar
  17. Bradley, W. G.,and Jenkinson, M., 1975, Neural abnormalities in the dystrophic mouse, J. Neurol. Sci 25: 249.Google Scholar
  18. Bradley, W. G., Jams, E., and Jenkinson, M., 1977, The nodes of Ranvier in the nerves of mice with muscular dystrophy, J. Neuropathol. Exp. Neurol 36: 797.PubMedCrossRefGoogle Scholar
  19. Braverman, I. M., 1953, Neurological actions caused by the mutant gene “trembler” in the house mouse: An investigation, J. Neuropathol. Exp. Neurol 12: 64.PubMedCrossRefGoogle Scholar
  20. Bray, G. M., Rasminsky, M., and Aguayo, A. J., 1981, Interactions between axons and their sheath cells, Annu. Rev. Neurosci 4: 127.PubMedCrossRefGoogle Scholar
  21. Brenkert, A., Arora, R. C., Radin, N. S., Meier, H., and MacPike, A. D., 1972, Cerebroside synthesis and hydrolysis in a neurological mutant mouse (MSD), Brain Res. 36: 195.PubMedCrossRefGoogle Scholar
  22. Brostoff, S. W., Powers, J., Wisniewski, H. M., and Hogan, E. I., 1975, Encepha1itogenic activity of quaking mutant mouse myelin, Trans. Am. Soc. Neurochem 6: 208.Google Scholar
  23. Brostoff, S. W., Greenfield, S., and Hogan, E. L., 1977, The differentiation of synthesis from incorporation of basic protein in quaking mutant mouse myelin. Brain Res. 120: 517.PubMedCrossRefGoogle Scholar
  24. Bunge, R. P., and Bunge, M. B., 1978, Evidence that contact with connective tissue matrix is required for normal interaction between Schwann cells and nerve fibers, J. Cell Biol 78: 943.PubMedCrossRefGoogle Scholar
  25. Bunge, R. P., Bunge, M. B., Okada, E., and Cornbrooks, C., 1980, Abnormalities expressed in cultures prepared from peripheral nerve tissues of trembler and dystrophic mice, in: Neurological Mutations Affecting Myelinat ion (N. Baumann, ed.), INSERM Symposium No. 14, pp. 433 - 446, Elsevier/ North-Holland, Amsterdam.Google Scholar
  26. Burkart, T., Wiesmann, V. N., Siegrist, H. P., and Herschkowitz, N. N., 1981, Net sulfatide synthesis,Google Scholar
  27. Greenfield, S., Brostoff, S., and Hogan, E. L., 1977, Evidence for the defective incorporation of proteins in myelin of the quaking mutant mouse Brain Res. 120:507 Google Scholar
  28. Greenfield, S., Williams, N. I., White, M., Brostoff, S. W., and Hogan, E. L., 1979, Proteolipid protein: Synthesis and assembly into quaking mouse myelin J. Neurochem. 32:1647 Google Scholar
  29. Greenfield, S., Brostoff, S. W., and Hogan, E. L., 1980, Characterization of the basic proteins from rodent peripheral nervous system myelin J. Neurochem. 34:453 Google Scholar
  30. Gregson, N. A., and Oxberry, J. M., 1972, The composition of myelin from the mutant mouse “quaking” J. Neurochem. 19:1065 Google Scholar
  31. Hack, M. H., and Helmy, F. M., 1978, The diminution of the myelin ethanolamine plasmalogen in brain of the jimpy mouse and brain and spinal cord of the quaking mouse as visualized by thin-layer chromatography, J. Chromatogr. 145: 307.PubMedCrossRefGoogle Scholar
  32. Hamburgh, M., and Bornstein, M. K., 1970, Myelin synthesis in two demyelinating mutations in mice Exp. Neurol. 28:471 Google Scholar
  33. Harris, J. B., and Wilson, P., 1971, Denervation murine dystrophy, Nature (London) 229:61. Harrison, B. M., 1981, Autonomic neuropathy in the trembler mouse, J. Autonom. Nerv. Sys. 4:393. Hatfield, J. S., and Skoff, R. P., 1982, GFAP immunoreactivity reveals astrogliosis in females heterzygous for jimpy, Brain Res. 250: 123.Google Scholar
  34. Hauser, G., Eichberg, J., and Jacobs, S., 1971, Polyphosphoinositide levels and biosynthesis in mice Exp. Neurol,28:471 Google Scholar
  35. Herschkowitz, N., Vassella, F., and Bischoff, A., 1971, Myelin differences in the central and peripheral nervous system in the “jimpy” mouse J. Neurochem. 18:1361 Google Scholar
  36. Hertz, L., Chaban, G., and Hertz, E., 1980, Abnormal metabolic response to excess potassium in astrocytes from the jimpy mouse; a convulsing neurological mutant, Brain Res. 181: 482.PubMedCrossRefGoogle Scholar
  37. Hof, L., and Csiza, C. K., 1982, Developmental changes in glycolipid synthesizing enzymes in the brain of a myelin-deficient mutant Wistar rat, J. Neurochem 39: 1434.PubMedCrossRefGoogle Scholar
  38. Hogan, E. L., and Joseph, K. C., 1970, Composition of cerebral lipids in murine leucodystrophy: The quaking mutant J. Neurochem. 17:1209 Google Scholar
  39. Hogan, E. L., Joseph, K. C., and Schmidt, G., 1970, Composition of cerebral lipids in murine sudanophilic leukodystrophy: The jimpy mutant J. Neurochem. 17:75 Google Scholar
  40. Hogan, E. L., Greenfield, S. and Brostoff, S. W., 1979, Genetic disturbance of myelinogenesis in the mouse, in: Neurogenetics: Genetic Approaches to the Nervous System (X. Breakefield, ed.), pp. 153 - 172, Elsevier, New York.Google Scholar
  41. Howell, J. MeC., 1970, Diseases affecting myelmation in domestic animals, in: Myelination (A. M. Davison and A. Peters, eds.), pp. 199 - 228, Charles C Thomas, Springfield, Illinois.Google Scholar
  42. Hughes, L. E., Kershaw, G. F., and Shaw, I. G., 1959, ’B" or border disease: An undescribed disease of sheep, Yet. Rec. 71:313.Google Scholar
  43. Huizar, P., Kuno, M., and Mizata, Y., 1975, Electrophysiological properties of spinal motor neurons of normal and dystrophic mice, J. Physiol. (London) 248: 231.Google Scholar
  44. Igisu, H., Takahashi, H., Suzuki, K., and Suzuki, K., 1983, Abnormal accumulation of galactosyl- ceramide in the kidney of twitcher mouse, Biochem. Biophys. Res. Commun 110 (3): 940.PubMedCrossRefGoogle Scholar
  45. Inoue, Y., Nakamura, R., Mikoshiba, K., and Tsukoda, Y., 1981, Fine structure of the central myelin sheath in the myelin deficient mutant shiverer mouse, with special reference to the pattern of myelin formation by oligodendroglia, Brain Res. 219: 85 - 94.PubMedCrossRefGoogle Scholar
  46. Jablecki, C., and Brimijoin, S., 1974, Reduced axoplasmic transport of choline acetyltransferase activity in dystrophic mice Nature (London) 250:151-154 Google Scholar
  47. Jacobs, J. M., Scaravilli, F., and DeAranda, F. T., 1982, The pathogenesis of globoid cell leucodystrophy in peripheral nerve of the mouse mutant twitcher, J. Neurol. Sei 55: 285.CrossRefGoogle Scholar
  48. Jacque, C. M., Harpin, M. I., and Baumann, N. A., 1969, Brain lipid analysis of a myelin deficient mutant, the “quaking” mouse Eur. J. Biochem. 11:218 Google Scholar
  49. Jacque, C. M., Jorgensen, O. S., and Bock, E., 1974, Quantitative studies of the brain specific antigens S-100, GFA, 14-3-2, Dl, D2, D3, and Cl in quaking mouse FEBS Lett. 49:264 Google Scholar
  50. Jacque, M., Baumann, A., and Bock, E., 1976, Quantitative studies of the brain specific antigens GFA, 14-3-2, synaptin Cl, D1, D2, D3, and D5 in jimpy mouse, Neurosci. Lett 3: 41.PubMedCrossRefGoogle Scholar
  51. Jacque, C., Lachapelle, F., Collier, P., Raoul, M., and Baumann, N., 1980, Accumulation of GFA, the monomeric precursor of the gliofilaments, during development in normal mice and dysmyelinating mutants, J. Neurosci. Res. 5: 379.Google Scholar
  52. Jaros, E., and Bradley, W. G., 1979, Atypical axon-Schwann cell relationships in the common peronealGoogle Scholar
  53. nerve of the dystrophic mouse: An ultrastructural study, Neuropathol. Exp. Neurobiol. 5:133. Jortner, B. S., and Jonas, A. M., 1968, The neuropathology of globoid cell leucodystrophy in the dog, Acta Neuropathol. 10: 171.Google Scholar
  54. Joseph, K. C., and Hogan, E. L., 1971, Fatty acid composition of cerebrosides, sulfatides and ceramides in murine sudanophilic leucodystrophy: The jimpy mutant, J. Neurochem 18: 1639.PubMedCrossRefGoogle Scholar
  55. Joseph, K. C., Druse, M. J., Newell, L. R., and Hogan, E. L., 1972, Fatty acid composition of cerebrosides, sulphatides and ceramides in murine leucodystrophy: The quaking mutant, J. Neurochem 19: 307PubMedCrossRefGoogle Scholar
  56. Jutzi, H., Siegrist, H. P., Burkart, T., Wiesmman, U., and Herschkowitz, N. N., 1979, Diminished cerebroside sulfotransferase activity in the jimpy mouse mutant due to altered lipid composition in microsomal membranes, Biochim. Biophys. Acta 552: 413.PubMedCrossRefGoogle Scholar
  57. Kandutsch, A. A., and Saucier, S. E., 1969, Regulation of sterol synthesis in developing brains of normal and jimpy mice, Arch. Biochem. Biophys 135: 201.PubMedCrossRefGoogle Scholar
  58. Kandutsch, A. A., and Saucier, S. E., 1972, Sterol and fatty acid synthesis in developing brains of three myelin deficient mouse mutants, Biochim. Biophys. Acta 260: 26.PubMedCrossRefGoogle Scholar
  59. Kanfer, J., and Stein, M., 1972, Sulfatide biosynthesis by intact microsomes and Triton extracts of normal and “quaking” mouse brain, Lipids 7: 259.PubMedCrossRefGoogle Scholar
  60. Keen, C. L., and Hurley, L. S., 1976, Copper supplementation in quaking mutant mice: Reduced tremors and increased brain copper, Science 193: 244.PubMedCrossRefGoogle Scholar
  61. Kelton, D. E., and Rauch, H., 1962, Myelination and myelin degeneration in the CNS of dilute-lethal mice, Exp. Neurol. 6: 252.PubMedCrossRefGoogle Scholar
  62. Kempf, E., Greilsamer, J., Mack, G., and Mandel, P., 1973, Turnover of brain adrenergic transmitters in quaking mice, J. Neurochem 20: 1269.PubMedCrossRefGoogle Scholar
  63. Kirschner, P. A., and Ganser, A. L., 1980, Compact myelin exists in the absence of basic protein in the shiverer mutant mouse, Nature (London) 283: 207.CrossRefGoogle Scholar
  64. Kirschner, D. A., and Sidman, R. L., 1976, X-ray diffraction study of myelin structure in immature and mutant mice, Biochim. Biophys. Acta 448: 73.PubMedCrossRefGoogle Scholar
  65. Kishimoto, Y., 1971, Abnormality in sphingolipid fatty acids from sciatic nerve and brain of quaking mice, J. Neurochem 18: 1365.PubMedCrossRefGoogle Scholar
  66. Kobayashi, T., Yamanaka, T., Jacobs, J. M., Teixeira, F., and Suzuki, K., 1980, The twitcher mouse: An enzymatically authentic model of human globoid cell leukodystrophy (Krabbe disease), Brain Res. 202: 479.PubMedCrossRefGoogle Scholar
  67. Kodama, S., Igisu, H., Siegel, D. A., and Suzuki, K., 1982, Glycosylceramide synthesis in the developing spinal cord and kidney of the twitcher mouse, an enzymatically authentic model of human Krabbe disease, J. Neurochem. 39:1314,Google Scholar
  68. Kohlschutter, A., and Herschkowitz, N. H., 1973, Sulfatide synthesis in neurons: A defect in mice with a hereditary myelination disorder, Brain Res. 50: 379.PubMedCrossRefGoogle Scholar
  69. Komiya, Y., and Austin, L., 1974, Axoplasmic flow of protein in the sciatic nerve of normal and dystrophic mice, Exp. Neurol 43: 1.PubMedCrossRefGoogle Scholar
  70. Kostic, D., Nussbaum, J. L., and Mandel, P., 1969, A study of brain ganglioside in jimpy mutant mice, Life Sci. 8: 1135.PubMedCrossRefGoogle Scholar
  71. Kostic, D., Nussbaum, J. L., and Neskovic, N., 1970, Les gangliosides du cerveau de deux mutants “quaking” et “jimpy,” Acta Med. lu,eosl 24: 20.Google Scholar
  72. Kraus-Ruppert, R., Herschkowitz, N., and Furst, S., 1973, Morphological studies on neuroglial cells in the corpus callosum of the jimpy mutant mouse, J. Neuropathol. Exp. Neurol 32: 197.PubMedCrossRefGoogle Scholar
  73. Kristt, D. A., and Butler, F. K., 1978. Neuronal abnormalities associated with impaired myelination during brain development: A Golgi study of neocortex in the jimpy mouse, Neuroscience Lett. 7: 107.CrossRefGoogle Scholar
  74. Kurihara, T., Nussbaum, J. L., and Mandel, P., 1969, 2’,3’-Cyclic nucleotide 3’-phosphohydrolase in the brain of the jimpy mouse, a mutant with deficient myelination, Brain Res. 13: 401.Google Scholar
  75. Kurihara, T., Nussbaum, J. L., and Mandel, P., 1970, 2’,3’-Cyclic nucleotide 3’-phosphohydrolase in brains of mutant mice with deficient myelination, J. Neurochem 17: 993.Google Scholar
  76. Kurihara, T., Nussbaum, J. L., and Mandel, P., 1971, 2’,3’-Cyclic nucleotide 3’-phosphohydrolase in purified myelin from brain of jimpy and normal young mice, Life Sci. 10: 421.Google Scholar
  77. Kurtz, D. J., and Kanfer, J. N., 1970, Cerebral acid hydrolase activities: Comparison in quaking and normal mice, Science 168: 259.PubMedCrossRefGoogle Scholar
  78. Larrouquere-Regnier, S., Boiron, S., Darriet, P., Cassagne, C., and Bourre, J. M., 1979, Lipid composition of sciatic nerve from dysmyelinating trembler mouse, Neurosci. Lett 15: 135.PubMedCrossRefGoogle Scholar
  79. Lerner, P., Campognoni, A. G., and Sampugna, J., 1974, Proteolipids in the developing brains of normal mice and myelin deficient mutants, J. Neurochem 22: 163.PubMedCrossRefGoogle Scholar
  80. Low, P. A., 1976a, Hereditary hypertrophie neuropathy in the trembler mouse. Part 1. Histopathological studies: Light microscopy, J. Neurol. Sci 30: 327.PubMedCrossRefGoogle Scholar
  81. Mikoshiba, K., Kohsaka, S., Takamatsu, K., andisukada, 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.PubMedCrossRefGoogle Scholar
  82. Mithcn, F. A., Cochran, M., Cornbrooks, C. J., and Bunge, R. P., 1982, Expression of the trembler mouse mutation in organotypic cultures of dorsal root ganglia, Dez. Brain Res 4: 407.CrossRefGoogle Scholar
  83. Morel!, P., and Costantino-Ceccarini, E., 1972, Jimpy mouse lu vitro studies of brain sphingolipid biosynthesis Lipids 7:266 Google Scholar
  84. Murad, S., and Kishimoto, Y., 1975, a-Hydroxylation of lignoceric acid to cerebronic acid during brain development: Diminished hydroxylase activity in myelin-deficient mouse mutants, J. Biol. Chem 250: 5841.Google Scholar
  85. Nagaike, K., Mikoshiba, K., and Tsukada, T., 1982, Dysmyelination of shiverer mutant mice in vino and in vitro J. Neurochem. 39:1235 Google Scholar
  86. Nagara, H., and Suzuki, K., 1982, Radial component of the central myelin in neurologic mutant mice Lab. Inzest. 47:51 Google Scholar
  87. Nagara, H., Kobayashi, T., Suzuki, K., and Suzuki, K., 1982, The twitcher mouse: Normal pattern of early myelination in the spinal cord, Brain Res. 244: 289.PubMedCrossRefGoogle Scholar
  88. Neskovic, N. M., Nussbaum, J. L., and Mandel, P., 1970, A study of glycolipid metabolism in myelination disorder of jimpy and quaking mice, Brain Res. 21: 39.PubMedCrossRefGoogle Scholar
  89. Neskovic, N. M., Sarlieve, L. L., and Mandel, P., 1972, Biosynthesis of glycolipids in myelin deficient mutants: Brain glycosyl transf erase in jimpy and quaking mice, Brain Res. 42:147.Google Scholar
  90. Norton, W. T., and Poduslo, S. E., 1973, Myelination in rat brain: Method of myelin isolation, J. Neurochem. 21: 749.PubMedCrossRefGoogle Scholar
  91. Nussbaum, J. L., and Mandel, P., 1972, Enzymic synthesis of psychosine sulphate, J Neurochem. 13:1789 Google Scholar
  92. Nussbaum, J. L., and Mandel, P., 1973, Brain proteolipids in neurological mutant mice Brain Re.s. 61:295 Google Scholar
  93. Nussbaum, J. L., Neskovic, N., and Mandel, P., 1969, A study of lipid components in brain of the “jimpy” mouse, a mutant with myelin deficiency, J. Neurochem 16: 927.PubMedCrossRefGoogle Scholar
  94. Nussbaum, J. L., Neskovic, N., and Mandel, P., 1971, The fatty acid composition of phospho]ipids and glycolipids in jimpy mouse brain, J.Neurochem 18: 1529.PubMedCrossRefGoogle Scholar
  95. Okada, E., Mizuhira, V., and Nakamura, H., 1976, Dysmyelination in the sciatic nerves of dystrophic mice, J. Neurol. Sci 28: 505.PubMedCrossRefGoogle Scholar
  96. Okada, E., Bunge, R. P., and Bunge, M. B., 1980, Abnormalities expressed in Tong-term cultures of dorsal root ganglia from the dystrophic mouse, Brain Res. 194: 455.PubMedCrossRefGoogle Scholar
  97. Olafson, R. W., Drummond, G. I., and Lee, J. F., 1969, Studies on 2’,3’-cyclic nucleotide-3’phosphohydrolase from brain, Can. J. Biochem 47: 961.PubMedCrossRefGoogle Scholar
  98. Omlin, F. X., Bischoff, A., Spycher, M. A., and Wiesmann, V. N., 1979, Ultrasu-ucture of lipids in the optic nerve of the mouse mutant “jimpy” Acta Neuropathol. 45: 221.Google Scholar
  99. Omlin, F. X., Bischoff, A., and Moor, H., 1980, Myelin and glial membrane structures in the optic nerve of normal and jimpy mouse, J. Neuropathol. Exp. Neurol 39: 215.PubMedCrossRefGoogle Scholar
  100. Patterson, D. S. P., Terlecki, S., Foulkes, J. A., Sweasey, D., and Glancy, E. M., 1975, Spinal cord lipids and myelin composition in border disease (hypomyClinogenesis eongenita) of lambs, J. Neurochem 24: 513.PubMedCrossRefGoogle Scholar
  101. Perkins, C. S., Aguayo, A. J., and Bray, G. M., 1980a, Expression of the trembler gene requires Schwann cells being challenged to myclinate, Neurology 30: 408.Google Scholar
  102. Perkins, S. C., Aguayo, A. J., and Bray, G. M., 19801), Evidence for undifferentiated Schwann cells in the spinal roots of dystrophic mice, Can. J. Neurol. Sci 7: 123.Google Scholar
  103. Perkins, C. S., Aguayo, A. J., and Bray, G. M., 1981, Schwann cell multiplication in Trembler mice Neuropathol Appl. Neurohiol. 7:115 Google Scholar
  104. Peterson, A., 1979, Mosaic analysis of dystrophic and normal chimeras: An approach to mapping the site of gene expression, Ann. N. Y. Acad. Sci 317: 629.Google Scholar
  105. Phillips, J. S. R., 1954, Jimpy: A new totally sex-linked gene in the house mouse Arch. Indukt. Abstamnzungs-l’ererbungsl. 86:322 Google Scholar
  106. Plant, J. W., Acland, H. M., and Gard, G. P., 1974, Mucosal disease virus infection of sheep N. S. IV. Pet. Yroc. 1:38 Google Scholar
  107. Pollard, J. D., and McLeod, J. G., 1980, Nerve grafts in the trembler mouse: An electrophysiological and histological study, J.Veuroi.ci. 46: 373.Google Scholar
  108. Pol let, S., Cha ix, G., and Baumann, N., 2976, Calcium cnit ten tof mice brain lipids during myelination, Neurosci. Lett 3: 311.Google Scholar
  109. Privat, A., Robain, O., and Mandel, P., 1972, Aspect ultrastructuraux du corps calleux chez la souris Jimpy, Acta Veuropa1hol. 21: 282.CrossRefGoogle Scholar
  110. Privat, A., Drian, M. J., and Escaig, J., 1979a, Jimpy mouse myelin revisited with freeze-fracture, Acta Neuropathol. 45: 129.PubMedCrossRefGoogle Scholar
  111. Privat, A., Jacque, C., Bourre, J. M., Dupouey, P., and Baumann, N., 1979b, Absence of the major dense line in myelin of the mutant mouse Shiverer, Neurosci. Lett 12: 107.PubMedCrossRefGoogle Scholar
  112. Prohaska, J., 1980, Normal copper metabolism in quaking mice, Life Sci. 26: 731.PubMedCrossRefGoogle Scholar
  113. Rasminsky, M., Kearing, R. E., Aguayo, A. J., and Bray, G. M., 1978, Conduction of nervous impulses in spinal roots and peripheral nerves of dystrophic mice, J. Neural. Sei 143: 71.Google Scholar
  114. Rauch, H., and Yost, M. T., 1963, Phenylalanine metabolism in dilute-lethal mice, Genetics 48: 1487.PubMedGoogle Scholar
  115. Rayburn, H. R., Peterson, A. C., and Aguayo, A. J., 1980, Development and deployment of SchwannGoogle Scholar
  116. cells in peripheral nerves of trembler and normal mouse chimeras, Soc. Neurosci. Abstr 6:660.Google Scholar
  117. Roach, A., Boylan, K., Horvath, S., Prusiner, S. B., and Hood, L. E., 1983, Characterization of cloned cDNA representing rat myelin basic protein: Absence of expression in brain of shiverer mice, Cell 34: 799.PubMedCrossRefGoogle Scholar
  118. Robain, O., and Mandel, P., 1974, Etude quantitative de la myelination et de la croissance axonale dansGoogle Scholar
  119. le corps calleux et le cordon posterieur de la moelle chez la souris Jimpy, Acta Neuropathol 29:293.Google Scholar
  120. Rosenbluth, J., 1980a, Central myelin in the mouse mutant shiverer, J. Comp. Neurol 194: 639.PubMedCrossRefGoogle Scholar
  121. Rosenbluth, J., 1980b, Peripheral myelin in the mouse mutant shiverer, J. Comp. Neurol. 193: 729.Google Scholar
  122. Rosenbluth, J., 1981, Axoglial junctions in the mouse mutant shiverer, Brain Res. 208: 283.PubMedCrossRefGoogle Scholar
  123. Ruenwongsa, P., Singh, H., and Jungalwala, F. B., 1979, Protein-catalyzed exchange of phosphatidyl inositol between rat brain microsomes and myelin, J. Biol. Chem 254: 9385.PubMedGoogle Scholar
  124. Russell, D. H., and Meier, H., 1975, Alterations in the accumulation patterns of polyamines in brains of myelin-deficient mice, J. Neurobiol 6: 267.PubMedCrossRefGoogle Scholar
  125. Salafsky, B., and Stirling, C. A., 1973, Altered neural protein in murine muscular dystrophy, Nature (London) New Biol. 246: 126.CrossRefGoogle Scholar
  126. Samorajski, T., Freide, R. L., and Reimer, P. R., 1970, Hypomyelination in the quaking mouse: A model for the analysis of disturbed myelin formation, J. Neuropathol. Exp. Neurol 29; 507.PubMedCrossRefGoogle Scholar
  127. Saperstein, V. S., Flynn, C., and Lees, M. B., 1980, Developmental changes in carbonic anhydrase and adenylate cyclase in quaking mice, Brain Res. 185: 373.CrossRefGoogle Scholar
  128. Sarlieve, L. L., Neskovic, N. M., and Mandel, P., 1971, PAPS-cerebroside sulfotransferase activity in brain and kidney of neurological mutants, FEBS Lett. 19: 91.PubMedCrossRefGoogle Scholar
  129. Sarlieve, L. L., Neskovic, N. M., Rebel, G., and Mandel, P., 1972, Some properties of brain PAPScerebroside sulphotransferase in jimpy and quaking mice, Neurobiology 2: 70.PubMedGoogle Scholar
  130. Sarlieve, I. L., Neskovic, N. M., Rebel, G., and Mandel, P., 1974, PAPS-cerebroside sulphotransferaseGoogle Scholar
  131. activity in developing brain of a neurological mutant of mouse (MSD), Exp. Brain Res. 19:158. Sarlieve, L. I,., Farooqui, A. A., Rebel, G., and Mandel, P., 1978, Arylsulphatase A and 2’,3’-cyclic nucleotide, 3’-phosphohydrolase activities in the brains of myelin deficient mice, Neuroscience 1: 519.Google Scholar
  132. Scaravilli, F., and Jacobs, J. M., 1981, Peripheral nerve grafts in hereditary leukodystrophy mutant mice (twitcher), Nature (London) 290: 56.CrossRefGoogle Scholar
  133. Scaravilli, F., and Jacobs, J. M., 1982, Improved myelination in nerve grafts from the leucodystrophic twitcher in trembler mice: Evidence for enzyme replacement, Brain Res. 237: 163.PubMedCrossRefGoogle Scholar
  134. Schneck, L., Adachi, M., and Volk, B. W., 1971, Congenital failure of myelinization: PelizaeusMerzbacher disease, Neurology 21: 817.PubMedCrossRefGoogle Scholar
  135. Shaw, I. G., Winkler, C. E., and Terlecki, S., 1967, Experimental reproduction of hypomyelinogenesis of lambs, Vet. Rec 8: 115.Google Scholar
  136. Sheads, L. D., Sampugna, J., and Campagnoli, A. T., 1974, Developmental changes of steryl esters in normal and jimpy mouse brain, J. Neurochem 22: 1149.PubMedCrossRefGoogle Scholar
  137. Sheads, I. D., Eby, M.,J., Sampugna, J., and Douglas, L. W., 1977, Myelin subtractions isolated from mouse brain: Studies of normal mice during development, quaking mutants and three brain regions, J. Neurobiol 8: 67.Google Scholar
  138. Sidman, R. I., Dickie, M. M., and Appel, S. H., 1964, Mutant mice (quaking and jimpy) with deficient myelination in the central nervous system, Science 144: 309.PubMedCrossRefGoogle Scholar
  139. Simler, S., Owsianowski, N., Randrianarisoa, H., Seydig, M., and Mandel, P., 1974, Biosynthesis of free amino acids in the brain of jimpy mice, J. Neurochem 23: 359.PubMedCrossRefGoogle Scholar
  140. Singh, H., Spritz, N., and Geyer, B., 1971, Studies of brain myelin in the “quaking mouse,” J. Lipid Res. 12: 473.PubMedGoogle Scholar
  141. Singh, I., Kishim oto, Y., 1982, Brain-specific ceramide synthesis activity: Change during brain maturation and in jimpy mouse brain, Brain Res. 232: 500.PubMedCrossRefGoogle Scholar
  142. Skoff, R. P., 1976, Myelin defecit in the jimpy mouse may be due to cellular abnormalities in astroglia,Google Scholar
  143. Zak, B., Pullet, S. A., Harpin, M. I., and Baumann, N. A., 1974, Ceramide biosynthesis in mouse brain microsontes: Comparison between C57B1 conuols and quaking mutants, Brain Res. 81:511. Zannoni, V. G., Weber, W. W., Van Valen, P., Rubin, A., Bernstein, R., and La Du, B. N., 1966,Google Scholar
  144. P lien via lanine metabolism and “phenylketonuria” in dilute-lethal mice, Genetics 54:1391. Zeller, N. K., Sprague, J. A., Lazzarini, R. A., Yu, Y.-T. and Campagnoni, A. T., 1983, eDNA cloningof mouse myelin basic protein„ Trans, Am. Soc. Neurochern 14:255.Google Scholar
  145. Zimtnerman, T. R., and Cohen, S. R., 1979, The distribution of myelin basic protein in subeellular fractions of developing j impy mouse brain, J Neurochem 32: 817.Google Scholar

Copyright information

© Springer Science+Business Media New York 1984

Authors and Affiliations

  • Edward L. Hogan
    • 1
  • Seymour Greenfield
    • 1
  1. 1.Medical University of South CarolinaCharlestonUSA

Personalised recommendations