Fine Structure of Hereditary Defects of the Central Nervous System in Mice

  • Doris Burda Wilson


Of all the laboratory animals that exhibit hereditary defects of the nervous system, the house mouse (Mus musculus) has proven to be one of the richest sources of material. Not only are there numerous inbred strains of mice, but the number and variety of neurological mutations continue to grow. The latter thus serve as useful models for analyzing comparable human disorders. In many instances it is not known whether the mutant condition in an animal represents a human defect until a thorough morphological and physiological study is made. Moreover, although some defects, such as exencephaly, are immediately obvious, others may first be detected only as a behavioral disturbance, such as ataxia. Still other neurological defects may not be at all obvious until various morphological and functional features of the nervous system are methodically observed and tested.


Purkinje Cell Granule Cell Mutant Mouse Neural Tube Retinal Degeneration 
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.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Abbott, U. K. 1967. Avian developmental genetics, in: Methods in Developmental Biology, F. H. Wilt and N. K. Wessells, eds. Crowell, New York, pp. 13–52.Google Scholar
  2. Aguirre, G. 1981. Criteria for development of animal models of diseases of the eye. Am. J. Pathol 105: S187–S196.Google Scholar
  3. Altman, F. 1964. The inner ear in genetically determined deafness. Acta Otolaryngol. Suppl 187: 5–39.Google Scholar
  4. Altman, P. L., and Katz, D. D. 1979. Inbred and Genetically Defined Strains of Laboratory Animals. Part I. Mouse and Rat. FASEB, Bethesda Google Scholar
  5. Anniko, M. 1982. Specific pathology of the stria vascularis in postnatal progressive genetic inner ear disorder. Hear. Res 6: 247–258.PubMedCrossRefGoogle Scholar
  6. Anniko, M., Sobin, A., and Wersall, J. 1980. Vestibular hair cell pathology in the shaker-2 mouse. Arch Otorhinolaryngol. 226: 45–50.PubMedCrossRefGoogle Scholar
  7. Bancroft, M., and Bellairs, R. 1975. Differentiation of the neural plate and neural tube in the young chick embryo: A study by scanning and transmission electron microscopy. Anat. Embryol 147: 309–335.PubMedCrossRefGoogle Scholar
  8. Berger, B. 1971. Quelques aspects ultrastructuraux de la substance blanche chez la souris quaking. Brain Res. 25: 35–53.PubMedCrossRefGoogle Scholar
  9. Billings-Gagliardi, S., Adcock, L. H., and Wolf, M. K. 1980a. Hypomyelinated mutant mice: Description of jp msdand comparison with jpand qkon their present genetic backgrounds. Brain Res. 194: 325–338.CrossRefGoogle Scholar
  10. Billings-Gagliardi, S., Adcock, L. H., Schwing, G. B., and Wolf, M. K. 1980b. Hypomyelinated mutant mice. II. Myelination in vitro. Brain Res 200: 135–150.CrossRefGoogle Scholar
  11. Blanks, J. C., Adinolfi, A. M., and Lolley, R. N. 1974. Photoreceptor degeneration and synapto- genesis in retinal-degenerative (rd)mice. J. Comp. Neurol 156: 95–106.PubMedCrossRefGoogle Scholar
  12. Bok, D., and Hall, M. O. 1971. The role of the pigment epithelium in the etiology of inherited retinal dystrophy in the rat. J. Cell Biol 49: 664–682.PubMedCrossRefGoogle Scholar
  13. Borit A., and Sidman, R. L. 1972. New mutant mouse with communicating hydrocephalus and secondary aqueductal stenosis. Acta Neuropathol 21: 316–331.PubMedCrossRefGoogle Scholar
  14. Bryan, J. H., Hughes, R. L., and Bates T. J. 1977. Brain development in hydrocephalic-poly dactyl, a recessive pleiotropic mutant in the mouse. Virchows Arch. Pathol. Anat 374: 205–214.PubMedGoogle Scholar
  15. Burns, R. P., and Feeney, L. 1975. Hereditary cataracts in deer mice (Peromyscus maniculatus). Am. J. Ophthalmol. 80: 370–378.PubMedGoogle Scholar
  16. Burnside, B. 1971. Microtubules and microfilaments in newt neurulation. Dev. Biol 26: 416–441.PubMedCrossRefGoogle Scholar
  17. Caddy, K. W., and Biscoe, T. J. 1975. Preliminary observations on the cerebellum in the mutant mouse lurcher. Brain Res. 91: 276–280.PubMedCrossRefGoogle Scholar
  18. Caddy, K. W., and Biscoe, T. J. 1979. Structural and quantitative studies of the normal C3H and lurcher mutant mouse. Roy. Soc. Phil. Trans 287: 167–201.CrossRefGoogle Scholar
  19. Caddy, K. W., and Sidman, R. L. 1981. Purkinje cells and granule cells in the cerebellum of the stumbler mutant mouse. Dev. Brain Res 1: 221–236.CrossRefGoogle Scholar
  20. Caley, D. W., Johnson, C., and Liebelt, R. A. 1972. The postnatal development of the retina in the normal and rodless CBA mouse: A light and electron microscopic study. Am. J. Anat 133: 179–212.PubMedCrossRefGoogle Scholar
  21. Carter-Dawson, L. D., LaVail, M. M., and Sidman, R. L. 1978. Differential effect of the rdmutation on rods and cones in the mouse retina. Invest. Ophthalmol 17: 489–498.Google Scholar
  22. Caviness, V. S., Jr. 1977. Reeler mutant mouse: A genetic experiment in developing mammalian cortex. Soc. Neurosci. Symp 2: 27–46.Google Scholar
  23. Caviness, V. S., Jr., and Yorke, C. H., Jr. 1976. Interhemispheric neocortical connections of the corpus callosum in the reeler mutant mouse: A study based on anterograde and retrograde methods. J. Comp. Neurol 170: 449–460.PubMedCrossRefGoogle Scholar
  24. Center, E. M. 1969. Morphology and embryology of duplicitas posterior mice. Teratology2: 377–388.PubMedCrossRefGoogle Scholar
  25. Copp, A. J., Seller, M. J., and Polani, P. E. 1982. Neural tube development in mutant (curly tail) and normal mouse embryos: The timing of posterior neuropore closure in vivoand in vitro. J. Embryol. Exp. Morphol. 69: 151–167.Google Scholar
  26. Cullen, M. J., and Kaiserman-Abramof, I. R. 1976. Cytological organization of the dorsal lateral geniculate nuclei in mutant anophthalmic and postnatally enucleated mice. J. Neurocytol 5: 407–424.PubMedCrossRefGoogle Scholar
  27. Dempsey, E. E., and Trasler, D. G. 1983. Early morphological abnormalities in splotch mouse embryos and predisposition to gene- and retinoic acid-induced neural tube defects. Teratology 28: 461–472.PubMedCrossRefGoogle Scholar
  28. Deol, M. S. 1964a. The abnormalities of the inner ear in kreisler mice. J. Embryol. Exp. Morphol 12: 475–490.Google Scholar
  29. Deol, M. S. 1964b. The origin of the abnormalities of the inner ear in dreher mice. J.Embryol. Exp. Morphol. 12: 727–733.Google Scholar
  30. Deol, M. S. 1966. Influences of the neural tube on the differentiation of the inner ear in the mammalian embryo. Nature 209: 219–220.PubMedCrossRefGoogle Scholar
  31. Deol, M. S. 1968. Inherited diseases of the inner ear in man in the light of studies on the mouse. J. Med. Genet 5: 127–158.CrossRefGoogle Scholar
  32. Deol, M. S. 1970. Mouse mutants with inner ear defects and their value in biomedical research, in: Les Mutants Pathologiques chez’l Animal, M. Sabourdy, ed. CNRS, Paris, pp. 279–292.Google Scholar
  33. Deol, M. S. 1980. Genetic malformations of the inner ear in the mouse and in man. Birth Defects. 16: 243–261.PubMedGoogle Scholar
  34. Derer, P. 1979. Evidence for the occurrence of early modifications in the ‘glia limitans’ layer of the neocortex of the reeler mutant mouse. Neurosci. Lett 13: 195–202.PubMedCrossRefGoogle Scholar
  35. Detwiler, S. R., and Van Dyke, R. H. 1950. The role of the medulla in the differentiation of the otic vesicle. J. Exp. Zool 113: 179–199.CrossRefGoogle Scholar
  36. Donahue, S. 1964. A relationship between fine structure and function of blood vessels in the central nervous system of rabbit fetuses. Am. J. Anat 115: 17–26.PubMedCrossRefGoogle Scholar
  37. Dowling, J. E., and Sidman, R. L. 1962. Inherited retinal dystrophy in the rat. J. Cell Biol 14: 73–109.PubMedCrossRefGoogle Scholar
  38. Dryden, R. J. 1980. Duplication of the spinal cord: A discussion of the possible embryogenesis of diplomyelia. Dev. Med. Child Neurol 22: 234–243.PubMedCrossRefGoogle Scholar
  39. Duchen, L. W., Eicher, E. M., Jacobs, J. M., Scaravilli, F., and Teixeira, F. 1980. A globoid cell type of leucodystrophy in the mouse: The mutant twitcher, in: Neurological Mutations Affecting Myelination, N. Baumann, ed. Elsevier North-Holland, New York, pp. 107–122.Google Scholar
  40. Edelman, G. M. 1983. Cell adhesion molecules. Science 219: 450–457.PubMedCrossRefGoogle Scholar
  41. Ernston, S., Lundquist, P. G., Wedenberg, E., and Wersall, J. 1969. Morphologic changes in vestibular hair cells in a strain of the waltzing guinea pig. Acta Otolaryngol. 67: 521–534.CrossRefGoogle Scholar
  42. Erway, L. C., Fraser, A. S., and Hurley L. S. 1971. Prevention of congenital otolith defect in pallid mutant mice by manganese supplementation. Genetics 67: 97–108.PubMedGoogle Scholar
  43. Essner, E., and Gorrin, G. 1979. An electron microscopic study of macrophages in rats with inherited retinal dystrophy. Invest. Ophthalmol 18: 11–25.Google Scholar
  44. Feeney-Burns, L., Burns, R. P., and Anderson, R. S. 1980. Ultrastructure and acid phosphatase activity in hereditary cataracts of deer mice. Invest. Ophthalmol 19: 777–788.Google Scholar
  45. Flock, A., Cheung, H., and Wersall, J. 1979. Pathological actin in vestibular hair cells of the waltzing guinea pig. Adv. Otorhinolaryngol 25: 12–16.PubMedGoogle Scholar
  46. Freeman, D. B. 1972. Surface modifications of neural epithelial cells during formation of the neural tube in the rat embryo. J. Embryol. Exp. Morphol 28: 437–448.PubMedGoogle Scholar
  47. Friedrich, V. L., Jr. 1974. The myelin deficit in quaking mice. Brain Res. 82: 168–172.PubMedCrossRefGoogle Scholar
  48. Friedrich, V. L., Jr. 1975. Hyperplasia of oligodendrocytes in quaking mice. Anat. Embryol147: 259–271.PubMedCrossRefGoogle Scholar
  49. Glover, R. A., D’Amato, C. J., and Hicks, S. P. 1984. A new cell surface relationship between neuroepithelial cells during rat neural tube development. Exp. Neurol 83: 199–203.PubMedCrossRefGoogle Scholar
  50. Goffinet, A. M. 1979. An early development defect in the cerebral cortex of the reeler mouse. Anat. Embryol 157: 205–216.PubMedCrossRefGoogle Scholar
  51. Green, E. L., ed. 1966. Biology of the Laboratory Mouse, McGraw-Hill, New York.Google Scholar
  52. Green, M. G. 1970. The developmental effects of congenital hydrocephalus (ch)in the mouse. Dev. Biol23: 585–608.PubMedCrossRefGoogle Scholar
  53. Green, M. C., ed. 1981. Genetic Variants and Strains of the Laboratory Mouse. Fischer, Stuttgart.Google Scholar
  54. Grüneberg, H. 1953. Genetical studies on the skeleton of the mouse. VII. Congenital hydrocephalus. J. Genet 51: 327–358.CrossRefGoogle Scholar
  55. Guillery, R. W. 1974. Visual pathways in albinos. Sci. Am230: 44–54.PubMedCrossRefGoogle Scholar
  56. Hall, J. C., Greenspan, R. J., and Harris, W. A. 1982. Genetic Neurobiology. MIT, Cambridge.Google Scholar
  57. Hamai, Y., and Kuwabara, T. 1975. Early cytologic changes of Fraser cataract: An electron microscopic study. Invest. Ophthalmol 14: 517–527.PubMedGoogle Scholar
  58. Hamai, Y., Fukui, H. N., and Kuwabara, T. 1974. Morphology of hereditary mouse cataract. Exp. Eye Res 18: 537–546.PubMedCrossRefGoogle Scholar
  59. Hamburgh, M. 1963. Analysis of the postnatal developmental effects of “reeler,” a neurological mutation in mice. Dev. Biol 8: 165–185.CrossRefGoogle Scholar
  60. Handel, M. A., and Roth, L. E. 1971. Cell shape and morphology of the neural tube: Implications for microtubule function. Dev. Biol 25: 78–95.PubMedCrossRefGoogle Scholar
  61. Hanna, R. B., Hirano, A., and Pappas, G. D. 1976. Membrane specializations of dendritic spines and glia in the weaver mouse cerebellum: A freeze-fracture study. J. Cell Biol 68: 403–410.PubMedCrossRefGoogle Scholar
  62. Herman, L., and Kauffman, S. L. 1966. The fine structure of the embryonic mouse neural tube with special reference to cytoplasmic microtubules. Dev. Biol 13: 145–162.PubMedCrossRefGoogle Scholar
  63. Herrup, K. and Mullen, R. J. 1979. Staggerer chimeras: intrinsic nature of Purkinje cell defects and implications for normal cerebellar development. Brain Res. 178: 443–457.PubMedCrossRefGoogle Scholar
  64. Herrup, K., and Wilczynski, S. L. 1982. Cerebellar cell degeneration in the leaner mutant mouse. Neuroscience 7: 2185–2196.PubMedCrossRefGoogle Scholar
  65. Hirano, A., and Dembitzer, H. M. 1975. The fine structure of staggerer cerebellum. J. Neuropathol. Exp. Neurol 34: 1–11.PubMedCrossRefGoogle Scholar
  66. Hirano, A., Sax, D. S., and Zimmerman, H. M. 1969. The fine structure of the cerebella of a model of an inherited leukodystrophy in jimpy mice and their “normal” litter mates. Trans. Am. Neurol. Assoc 94: 171–177.Google Scholar
  67. Huang, F. L., Russell, P., and Kuwabara, T. 1980. Fine structure of lentoid bodies derived from normal and cataractous mouse lenses. Exp. Eye Res 31: 535–541.PubMedCrossRefGoogle Scholar
  68. Inoue, Y., Nakamura, R., Mikoshiba, K., and Tsukada, 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
  69. Inoue, Y., Inoue, K., Terashima, T., Mikoshiba, K., and Tsukada, Y. 1983. Developmental changes of oligodendroglia in the posterior funiculus of “shiverer” mutant mouse spinal cord, with special reference to myelin formation. Anat. Embryol 168: 159–171.PubMedCrossRefGoogle Scholar
  70. Jacobson, A. G. 1978. Some forces that shape the nervous system. Zoon6: 13–21.Google Scholar
  71. Jurand A. 1974. Some aspects of the development of the notochord in mouse embryos. J. Embryol. Exp. Morphol32: 1–33.PubMedGoogle Scholar
  72. Kaiserman-Abramof, I. R. 1979. Quantitative comparison of spines in layer V neurons of the striate cortex in anophthalmic mutant and normal mice. Brain Res. 179: 385–389.PubMedCrossRefGoogle Scholar
  73. Kalter, H. 1968. Teratology of the Central Nervous System. University of Chicago, Chicago.Google Scholar
  74. Kalter, H. 1980. A compendium of the genetically induced congenital malformations of the house mouse. Teratology 21: 397–429.PubMedCrossRefGoogle Scholar
  75. Kikuchi, K., and Hilding, D. A. 1965. The defective organ of Corti in shaker-1 mice. Acta Otolaryngol. 60: 287–303.CrossRefGoogle Scholar
  76. 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: 479–483.PubMedCrossRefGoogle Scholar
  77. Koniecki, D. L., and Friedrich, V. L., Jr. 1980. Ultrastructural alterations in synaptic boutons of quaking mice: Dense clusters of small vesicles. Brain Res. 199: 207–213.PubMedCrossRefGoogle Scholar
  78. Kuwamura, K., McLone, D. G., and Raimondi, A. J. 1978. The central (spinal) canal in congenital murine hydrocephalus: Morphological and physiological aspects. Child. Brain 4: 216–234.Google Scholar
  79. Lagenaur, C., Sommer, I., and Schachner, M. 1980. Subclass of astroglia in mouse cerebellum recognized by monoclonal antibody. Dev. Biol 79: 367–378.PubMedCrossRefGoogle Scholar
  80. Lai, Y. L., Jacoby, R. P., Jensen, J. T., and Yao, P. C. 1980. Retinitis pigmentosa. Animal model: Hereditary retinal degeneration in Wag/Rig rats. Am. J. Pathol 98: 281–284.PubMedGoogle Scholar
  81. Landis, S. C. 1973. Ultrastructural changes in the mitochondria of cerebellar Purkinje cells of nervous mutant mouse. J. Cell Biol 57: 782–797.PubMedCrossRefGoogle Scholar
  82. Landis, S. C. 1975. Histochemical demonstration of mitochondrial dehydrogenases in developing normal and nervous mutant mouse Purkinje cells. J. Histochem. Cytochem 23: 136–143.PubMedCrossRefGoogle Scholar
  83. Landis, D. M., and Landis, S. C. 1978. Several mutations in mice that affect the cerebellum. Adv.Neurol 21: 85–105.PubMedGoogle Scholar
  84. Landis, S. C., and Mullen, R. J. 1978. The development and degeneration of Purkinje cells in pcdmutant mice. J. Comp. Neurol 177: 125–143.PubMedCrossRefGoogle Scholar
  85. Landis, D. M., and Reese, T. S. 1977. Structure of the Purkinje cell membrane in staggerer and weaver mutant mice. /. Comp. Neurol 171: 247–260.CrossRefGoogle Scholar
  86. Landis, D. M., and Sidman, R. L. 1978. Electron microscopic analysis of postnatal histogenesis in the cerebellar cortex of staggerer mutant mice. J. Comp. Neurol 179: 831–864.PubMedCrossRefGoogle Scholar
  87. Landrieu, P., and Goffinet, A. 1981. Inverted pyramidal neurons and their axons in the neocortex of reeler mutant mice. Cell Tiss. Res 218: 293–301.CrossRefGoogle Scholar
  88. Lane, P. W., and Deol, M. S. 1974. Mocha, a new coat color and behavior mutation on chromosome 10 of the mouse. J. Hered 65: 362–364.PubMedGoogle Scholar
  89. Larsen, W. J., and Tung, H. N. 1978. Origin and fate of cytoplasmic gap junctional vesicles in rabbit granulosa cells. Tissue Cell 10: 585–598.PubMedGoogle Scholar
  90. La Vail, M. M. 1981. Analysis of neurological mutants with inherited retinal degeneration. Invest. Ophthalmol 21: 638–657.Google Scholar
  91. LaVail, M. M., and Mullen, R. J. 1976. Role of the pigment epithelium in inherited retinal degeneration analyzed with experimental mouse chimeras. Exp,. Eye Res 23: 227–245.CrossRefGoogle Scholar
  92. LaVail, M. M., Sidman, R. L., and O’Neil, D. 1972. Photoreceptor-pigment epithelial cell relationships in rats with inherited retinal degeneration: Radioautographic and electron microscope evidence for a dual source of extra lamellar material. J. Cell Biol 53: 185–209.CrossRefGoogle Scholar
  93. Lawson, R. F., and Raimondi, A. J. 1973. Hydrocephalus-3, a murine mutant. I. Alterations in fine structure of choroid plexus and ependyma. Surg. Neurol 1: 115–128.PubMedGoogle Scholar
  94. Leech, R. W., and Kohnen, P. 1974. Subependymal and intraventricular hemorrhages in the newborn. Am. J. Pathol 77: 465–474.PubMedGoogle Scholar
  95. Lim, D. J., and Erway, L. C. 1974. Influence of manganese on genetically defective otolith: A behavioral and morphological study. Ann. Otol. Rhinol. Laryngol 83: 565–581.PubMedGoogle Scholar
  96. Lim, D. J., Erway, L. C., and Clark, C. L. 1978. Tilted-head mice with genetic otoconial anomaly: Behavioural and morphological correlates, in: Vestibular Mechanisms in Health and Disease. J. H. Hood, ed. Academic, London, pp. 195–206.Google Scholar
  97. Lund, R. D. 1975. Variations in the laterality of the central projections of retinal ganglion cells. Exp. Eye. Res 21: 193–203.PubMedCrossRefGoogle Scholar
  98. Lund, R. D., Lund, J. S., and Wise, R. P. 1974. The organization of the retinal projection to the dorsal lateral geniculate nucleus in pigmented and albino rats. J. Comp. Neurol 158: 383–404.PubMedCrossRefGoogle Scholar
  99. Lyon, M. F. 1955. The developmental origin of hereditary absence of otoliths in mice. J. Embryol. Exp. Morphol3: 230–241.Google Scholar
  100. Lyon, M. F., and Meredith, R. 1969. Muted, a new mutant affecting coat colour and otoliths of the mouse, and its position in linkage group XIV. Genet. Res 14: 163–166.PubMedCrossRefGoogle Scholar
  101. Mariani, J., Crepel, F., Mikoshiba, K., Ghangeux, J. P., and Sotelo, C. 1977. Anatomical, physiological and biochemical studies of the cerebellum from reeler mutant mouse. Phil. Trans. R. Soc. Lond 281: 1–28.CrossRefGoogle Scholar
  102. Marin-Padilla, M. 1966. Mesodermal alterations induced by dimethyl sulfoxide. Proc. Soc. Exp. Biol. Med 122: 717–720.Google Scholar
  103. McLaughlin, B. J., and Boykins, L. G. 1981. Freeze-fracture study of photoreceptor outer segments and pigment epithelium in dystrophic and normal retinas. J. Comp. Neurol 199: 553–567.PubMedCrossRefGoogle Scholar
  104. McLone, D. G., Bondareff, W., and Raimondi, A. J. 1971. Brain edema in the hydrocephalic hy-3mouse: Submicroscopic morphology. J. Neuropathol. Exp. Neurol 30: 627–637.PubMedCrossRefGoogle Scholar
  105. McLone, D. G., Bondareff, W., and Raimondi, A. J. 1973. Hydrocephalus-3, a murine mutant. II. Changes in the brain extracellular space. Surg. Neurol 1: 233–242.PubMedGoogle Scholar
  106. Meier, C., and Bischoff, A. 1974. Dysmyelination in “jimpy” mouse: Electron microscopic study. J. Neuropathol. Exp. Neurol33: 343–353.CrossRefGoogle Scholar
  107. Meier, C., and Bischoff, A. 1977. Dysmyelination in jimpy mouse due to astroglial hyperplasia? Nature 268: 177.PubMedCrossRefGoogle Scholar
  108. Meier, H., and MacPike, A. D. 1970. A neurological mutation (msd)of the mouse causing a deficiency of myelin synthesis. Exp. Brain Res. 10: 512–525.PubMedCrossRefGoogle Scholar
  109. Meier, H., and MacPike, A. D. 1971. Three syndromes produced by two mutant genes in the mouse: Clinical, pathological, and ultrastructural bases of tottering, leaner, and heterozygous mice. J. Hered 62: 297–302.PubMedGoogle Scholar
  110. Meier, H., and MacPike, A. D. 1972. Myelin hypoplasia including peripheral neuropathy caused by three mutations of the mouse. Exp. Neurol 37: 643–646.PubMedCrossRefGoogle Scholar
  111. Meier, C., Herschkowitz, N., and Bischoff, A. 1974. Morphological and biochemical observations in the jimpy spinal cord. Acta Neuropathol. 27: 349–362.PubMedCrossRefGoogle Scholar
  112. Messer, A., and Smith, D. M. 1977. In vitrobehavior of granule cells from staggerer and weaver mutants of mice. Brain Res 130: 13–23.Google Scholar
  113. Mikaelian, D. P., and Ruben, R. J. 1964. Hearing degeneration in the shaker-1 mouse: Correlation of physiological observation with behavioral response and with cochlear anatomy. Arch. Otolaryngol 80: 418–430.PubMedGoogle Scholar
  114. Mikoshiba, K., Takamatsu, K., Kohsaka, S., Tsukada, Y., and Inoue, Y. 1982. Immunohistochemical and biochemical analyses of development of nervous system of mutant mice (reeler and shiverer), in: Genetic Approaches to Developmental Neurobiology, Y. Tsukada, ed. Springer, New York, pp. 195–221.Google Scholar
  115. Morris, G. L., and O’Shea, K. S. 1983. Anomalies of neuroepithelial cell associations in the splotch mutant embryo. Dev. Brain Res 9: 408–410.CrossRefGoogle Scholar
  116. Mullen, R. J. 1977. Genetic dissection of the CNS with mutant-normal mouse and rat chimeras. Soc. Neurosci. Symp 2: 47–65.Google Scholar
  117. Mullen, R. J., and La Vail, M. M. 1975. Two new types of retinal degeneration in cerebellar mutant mice. Nature 258: 528–530.PubMedCrossRefGoogle Scholar
  118. Nagara, H., and Suzuki, K. 1982. Radial component of the central myelin in neurologic mutant mice. Lab. Invest47: 51–59.PubMedGoogle Scholar
  119. Nagara, H., Yajima, K., and Suzuki, K. 1980. An ultrastructural study on the cerebellum of the brindled mouse. Acta Neuropathol. 52: 41–50.PubMedCrossRefGoogle Scholar
  120. 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: 289–294.PubMedCrossRefGoogle Scholar
  121. Nieuwkoop, P. D., and Weijer, C. J. 1978. Neural induction, a two-way process. Med. Biol 56: 366–371.PubMedGoogle Scholar
  122. Oda, S., Watanabe, K., Fujisawa, H., and Kameyaina, Y. 1980. Impaired development of lens fibers in genetic microphthalmia, eye lens obsolescence, Elo of the mouse. Exp. Eye Res 31:673–681. Google Scholar
  123. Pinto-Lord, M. C., and Caviness, V. S., Jr. 1979. Determinants of cell shape and orientation: A comparative Golgi analysis of cell-axon interrelationships in the developing neocortex of normal and reeler mice. J. Comp. Neurol 187: 49–70.PubMedCrossRefGoogle Scholar
  124. Povlishock, J. T., Martinez, A. J., and Mossy, J. 1977. The fine structure of blood vessels of the telencephalic germinal matrix in the human fetus. Am. J. Anat 149: 439–452.PubMedCrossRefGoogle Scholar
  125. Pratt, R. T. C. 1976. The Genetics of Neurological Disorders. Oxford University, New York.Google Scholar
  126. Privat, A., Drian, M. J., and Escaig, J. 1979. Jimpy mouse myelin revisited with freeze-fracture. Acta Neuropathol. 45: 129–131.PubMedCrossRefGoogle Scholar
  127. Privat, A., Valat, J., Lachapelle, F., Baumann, N., and Fulcrand, J. 1982. Radioautographic evidence for the protracted proliferation of glial cells in the central nervous system of jimpy mice. Dev. Brain Res 2: 411–416.CrossRefGoogle Scholar
  128. Putz, B., and Morriss-Kay, G. 1981. Abnormal neural fold development in trisomy 12 and trisomy 14 mouse embryos. I. Scanning electron microscopy. J. Embryol. Exp. Morphol66: 141–158.PubMedGoogle Scholar
  129. Rakic, P. 1976. Synaptic specificity in the cerebellar cortex: Study of anomalous circuits induced by single gene mutations in mice. Cold Spring Harbor Symp. Quant. Biol 40: 333–346.PubMedGoogle Scholar
  130. Rakic, P., and Sidman, R. L. 1973a. Weaver mutant mouse cerebellum: Defective neuronal migration secondary to abnormality of Bergmann glia. Proc. Natl. Acad. Sci. USA70: 240–244.CrossRefGoogle Scholar
  131. Rakic, P., and Sidman, R. L. 1973b. Sequence of developmental abnormalities leading to granule cell deficit in cerebellar cortex of weaver mutant mice. J. Comp. Neurol 152: 103–132.CrossRefGoogle Scholar
  132. Rakic, P., and Sidman, R. L. 1973c. Organization of cerebellar cortex secondary to deficit of granule cells in weaver mutant mice. J. Comp. Neurol152: 133–161.CrossRefGoogle Scholar
  133. Raviola, G., and Raviola, E. 1978. Intercellular junctions in the ciliary epithelium. Invest. Ophthalmol17: 958–981.Google Scholar
  134. Raviola, E., Goodenough, D. A., and Raviola, G. 1980. Structure of rapidly frozen gap junctions. J. Cell Biol 87: 273–279.PubMedCrossRefGoogle Scholar
  135. Revel, J. P., and Brown, S. S. 1976. Cell junctions in development, with particular reference to the neural tube. Cold Spring Harbor Symp. Quant. Biol 40: 433–455.Google Scholar
  136. Rezai, Z., and Yoon, C. H. 1972. Abnormal rate of granule cell migration in the cerebellum of “weaver” mutant mice. Dev. Biol 29: 17–26.PubMedCrossRefGoogle Scholar
  137. Robb, R. M. 1974. Electron microscopic histochemical studies of cyclic 3’, 5’-nucleotide phosphodiesterase in the developing retina of normal mice and mice with hereditary retinal degeneration. Trans. Am. Ophthalmol Soc 72: 650–669.PubMedGoogle Scholar
  138. Roffler-Tarlov, S., Beart, P. M., O’Gorman, S., and Sidman, R. L. 1979. Neurochemical and morphological consequences of axon terminal degeneration in cerebellar deep nuclei of mice with inherited Purkinje cell degeneration. Brain Res. 168: 75–95.PubMedCrossRefGoogle Scholar
  139. Rohrer, S. R., Shaw, S. M., and Lamar, C. H. 1978. Cadmium-induced endothelial cell alterations in the fetal brain from prenatal exposure. Acta Neuropathol. 44: 147–149.PubMedCrossRefGoogle Scholar
  140. Rosenbluth, J. 1979. Peripheral myelin in the mouse mutant shiverer. J. Comp. Neurol 193: 731–741.Google Scholar
  141. Rosenbluth, J. 1980. Central myelin in the mouse mutant shiverer. J. Comp. Neurol 194: 639–648.PubMedCrossRefGoogle Scholar
  142. Roy, S., Hirano, A., Kochen, J. A., and Zimmermann, H. M. 1974. Ultrastructure of cerebral vessels in chick embryo in lead intoxication. Acta Neuropathol. 30: 387–394.Google Scholar
  143. Rubin, R. J. 1973. Development and cell kinetics of the kreisler (kr/kr)mouse. Laryngoscope 83: 1440–1468.CrossRefGoogle Scholar
  144. Ruben, R. J. 1980. Pathogenesis of hereditary inner ear abnormalities in animals. Birth Defects 16: 29–34.PubMedGoogle Scholar
  145. Russell, P., Fukui, H. N., Tsunematsu, Y., Huang, F. L., and Kinoshita, J. H. 1977. Tissue culture of lens epithelial cells from normal and Nakano mice. Invest. Ophthalmol 16: 243–246.Google Scholar
  146. Sanyal, S., and Bal, A. K. 1973. Comparative light and electron microscopic study of retinal histogenesis in normal and rdmutant mice. Z. Anat. Entwicklungsgesch 142: 219–238.PubMedCrossRefGoogle Scholar
  147. Sanyal, S., and Hawkins, R. K. 1981. Genetic interaction in the retinal degeneration of mice. Exp. Eye Res33: 213–222.PubMedCrossRefGoogle Scholar
  148. Sanyal, S., and Jansen, H. G. 1981. Absence of receptor outer segments in the retina of rdsmutant mice. Neurosci. Lett 21: 23–26.PubMedCrossRefGoogle Scholar
  149. Sax. D. S., Hirano, A., and Shofer, R. J. 1968. Staggerer, a neurological murine mutant: An electron microscopic study of the cerebellar cortex in the adult. Neurology 18: 1093–1100.PubMedGoogle Scholar
  150. Schmidt, S. Y., and Lolley, R. N. 1973. Cyclic-nucleotide phosphodiesterase: An early defect in inherited retinal degeneration of C3H mice. J. Cell Biol 57: 117–123.PubMedCrossRefGoogle Scholar
  151. Seller, M. J., Embury, S., Polani, P. E., and Adinolfi, M. 1979. Neural tube defects in curly-tail mice. II. Effect of maternal administration of vitamin A. Proc. R. Soc. Lond. B206: 95–107.PubMedCrossRefGoogle Scholar
  152. Shiose, Y., and Sonohara, O. 1968. Studies on retinitis pigmentosa. XXVI. Electron microscopic aspects of early retinal changes in inherited dystrophic mice. Acta Soc. Ophthalmol. Jpn 72: 1126–1141.Google Scholar
  153. Shnerson, A., Lenoir, M., Van De Water, T. R., and Pujol, R. 1983. The pattern of sensorineural degeneration in the cochlea of the deaf shaker-1 mouse: Ultrastructural observations. Dev. Brain Res 9: 305–315.CrossRefGoogle Scholar
  154. Sidman, R. L. 1968. Development of interneuronal connections in brains of mutant mice, in: Physiological and Biochemical Aspects of Nervous Integration, F. D. Carlson, ed. Prentice-Hall, Englewood Cliffs, New Jersey, pp. 163–193.Google Scholar
  155. Sidman, R. L., and Green, M. C. 1965. Retinal degeneration in the mouse. J. Hered 56: 23–29.PubMedGoogle Scholar
  156. Sidman, R. L., Dickie, M. M., and Appel, S. H. 1964. Mutant mice (quaking and jimpy) with deficient myelination in the central nervous system. Science 144: 309–311.PubMedCrossRefGoogle Scholar
  157. Sidman, R. L., Green, M. C., and Appel, S. H. 1965. Catalog of the Neurological Mutants of the Mouse. Harvard University, Cambridge.Google Scholar
  158. Silver, J., and Robb, R. M. 1979. Studies on the development of the eye cup and optic nerve in normal mice and in mutants with congenital optic nerve aplasia. Dev. Biol68: 175–190.PubMedCrossRefGoogle Scholar
  159. Skoff, R. P. 1976. Myelin deficit in the jimpy mouse may be due to cellular abnormalities in astroglia. Nature 264: 560–562.PubMedCrossRefGoogle Scholar
  160. Skoff, R. P. 1982. Increased proliferation of oligodendrocytes in the hypomyelinated mouse mutant- jimpy. Brain Res. 248: 19–31.PubMedCrossRefGoogle Scholar
  161. Sobin, A., Anniko, M., and Flock, A. 1982. Rods of actin filaments in type I hair cells of the shaker- 2 mouse. Arch Otorhinolaryngol. 236: 1–6.PubMedCrossRefGoogle Scholar
  162. Sonohara, O., and Shiose, Y. 1968. Electron microscopic study of the visual cell of inherited retinal dystrophic mice. Folia Ophthalmol. Jpn 19: 77–86.Google Scholar
  163. Sotelo, C. 1973. Permanence and fate of paramembranous synaptic specializations in mutants and experimental animals. Brain Res. 62: 345–351.PubMedCrossRefGoogle Scholar
  164. Sotelo, C., and Changeux, J. P. 1974. Transsynaptic degeneration ’en cascade’ in the cerebellar cortex of staggerer mutant mice. Brain Res. 67: 519–526.PubMedCrossRefGoogle Scholar
  165. Sotelo, C., and Privat, A. 1978. Synaptic remodeling of the cerebellar circuitry in mutant mice and experimental cerebellar malformations: Study “in vivo”and “in vitro.” Acta Neuropathol 43: 19–34.PubMedGoogle Scholar
  166. Sotelo, C., and Triller, A. 1979. Fate of presynaptic afferents to Purkinje cells in the adult nervous mutant mouse: A model to study presynaptic stabilization. Brain Res. 175: 11–36.PubMedCrossRefGoogle Scholar
  167. Spiegelman, M., and Bennett, D. 1974. Fine structural study of cell migration in the early mesoderm of normal and mutant mouse embryos (TMocus: t g /t g ). J. Embryol. Exp. Morphol. 32: 723–738.Google Scholar
  168. Stein, K. F., and Rudin, I. A. 1953. Development of mice homozygous for the gene for looptail. J. Hered44: 59–69.Google Scholar
  169. Suzuki, K., and Zagoren, J. C. 1975. Focal axonal swelling in cerebellum of quaking mouse: Light and electron microscopic studies. Brain Res. 85: 38–43.PubMedCrossRefGoogle Scholar
  170. Takahashi, J., Hamai, M., and Mizuno, K. 1978. Morphometry of retinal mitochondria in hereditary retinal dystrophic mouse. Acta Soc. Ophthalmol. Jpn 82: 274–281.Google Scholar
  171. Takahashi, H., Igisu, H., Suzuki, K., and Suzuki, K. 1983. The twitcher mouse: An ultrastructural study on the oligodendroglia. Acta Neuropathol. 59: 159–166.PubMedCrossRefGoogle Scholar
  172. Tanaka, M., Russell, P., Smith, S., Uga, S., Kuwabara, T., and Kinoshita, J. H. 1980. Membrane alterations during cataract development in the Nakano mouse lens. Invest. Ophthalmol 19: 619–629.Google Scholar
  173. Theiler, K., Varnum, D. S., Nadeau, J. H., Stevens, L. C., Cagianut, B. 1976. A new allele of ocular retardation: Early development and morphogenetic cell death. Anat. Embryol 150: 85–97.PubMedGoogle Scholar
  174. Torii, J., Adachi, M., and Volk, B. W. 1971. Histochemical and ultrastructural studies of inherited leukodystrophy in mice. J. Neuropath. Exp. Neurol30: 278–289.PubMedCrossRefGoogle Scholar
  175. Trinkaus, J. P. 1978. Mediation of cell surface behavior by intercellular contact. Zoon 6: 51–63.Google Scholar
  176. Tripathi, R. C., and Ashton, N. 1976. Application of electron microscopy to the study of ocular inborn errors of metabolism. Birth Dejects 12: 69–104.Google Scholar
  177. Van Nie, R., Ivanyi, D., and Demant, P. 1978. A new H-2 linked mutation, rds, causing retinal degeneration in the mouse. Tiss. Antigen 12: 106–108.CrossRefGoogle Scholar
  178. Wahlsten, D. 1974. Heritable aspects of anomalous myelinated fibre tracts in the forebrain of the laboratory mouse. Brain Res. 68: 1–18.PubMedCrossRefGoogle Scholar
  179. Wahlsten, D. 1982. Deficiency of corpus callosum varies with strain and supplier of the mice. Brain Res. 239: 329–347.PubMedCrossRefGoogle Scholar
  180. Watanabe, I., and Bingle, G. J. 1972. Dysmyelination in “quaking” mouse: Electron microscopic study. J. Neuropathol. Exp. Neurol 31: 352–369.PubMedCrossRefGoogle Scholar
  181. Webster, W. S., and Briggs, D. 1981. A new mouse mutant showing cerebellar abnormalities. Brain Res. 218: 412–416.PubMedGoogle Scholar
  182. Wetts, R., and Herrup, K. 1983. Direct correlation between Purkinje and granule cell number in the cerebella of lurcher chimeras and wild-type mice. Dev. Brain Res 10: 41–47.CrossRefGoogle Scholar
  183. Wilson, D. B. 1976. Histological defects in the cerebellum of adult lurcher (Lc)mice. J. Neuropathol. Exp,. Neurol 35: 40–45.CrossRefGoogle Scholar
  184. Wilson, D. B. 1978. The fine structure of ventricular cells in the brains of mouse embryos homozygous for the loop-tail gene. Teratology 17:115–136.PubMedCrossRefGoogle Scholar
  185. Wilson, D. B. 1982. Cerebrovascular pathogenesis in the telencephalon of the loop-tail mouse: A transmission electron-microscopic study. Acta Neuropathol. 58: 177–182.PubMedCrossRefGoogle Scholar
  186. Wilson, D. B. 1983. Early development of the otocyst in an exencephalic mutant of the mouse. Acta Anat. 117: 217–224.PubMedCrossRefGoogle Scholar
  187. Wilson, D. B., and Finta, L. A. 1979. Gap junctional vesicles in the neural tube of the splotch (Sp)mutant mouse. Teratology 19: 337–340.PubMedCrossRefGoogle Scholar
  188. Wilson, D. B., and Finta, L. A. 1980a. Early development of the brain and spinal cord in dysraphic mice: A scanning electron microscopic study. Anat. Embryol 160: 315–326.CrossRefGoogle Scholar
  189. Wilson, D. B., and Finta, L. A. 1980b. Early development of the brain and spinal cord in dysraphic mice: A transmission electron microscopic study. J.Comp. Neurol.190: 363–371.PubMedCrossRefGoogle Scholar
  190. Wilson, D. B., and Michael, S. D. 1975. Surface defects in ventricular cells of the homozygous loop- tail brain: A scanning electron microscopic study. Teratology 11: 87–98.PubMedCrossRefGoogle Scholar
  191. Wilson, D. B., Finta, L. A., Center, E. M., and Paavola, L. G. 1982. An electron microscopic analysis of notochordal and mesenchymal cell abnormalities in embryos of Danforth’s short-tail (Sd)mice. Virchows Arch. Cell Pathol 39: 101–110.CrossRefGoogle Scholar
  192. Wilson, L., Sotelo, C., and Caviness, V. S., Jr. 1981. Heterologous synapses upon Purkinje cells in the cerebellum of the reeler mutant mouse: An experimental light and electron microscopic study. Brain Res. 213: 63–82.PubMedCrossRefGoogle Scholar
  193. Wisniewski, H., and Morell, P. 1971. Quaking mouse: Ultrastructural evidence for arrest of myelinogenesis. Brain Res. 29: 63–73.PubMedCrossRefGoogle Scholar
  194. Wozniak, M., McLone, D. G., and Raimondi, A. J. 1975. Micro- and macrovascular changes as the direct cause of parenchymal destruction in congenital murine hydrocephalus. J. Neurosurg 43: 535–545.PubMedCrossRefGoogle Scholar
  195. Wyse, J. P., and Hollenberg, M. J. 1977. Complicated colobomatous microphthalmos in the BW rat: A new form of inherited retinal degeneration. Am. J. Anat= 149: 377–411.PubMedCrossRefGoogle Scholar
  196. Yntema, C. L. 1950. An analysis of induction of the ear from foreign ectoderm in the salamander embryo. J. Exp. Zool 113: 211–243.CrossRefGoogle Scholar
  197. Yoon, C. H. 1972. Developmental mechanism for changes in cerebellum of “staggerer” mouse, a neurological mutant of genetic origin. Neurology 22: 743–754.PubMedGoogle Scholar
  198. Yoon, C. H., 1977a. Fine structure of the -cerebellum of “staggerer-reeler,” a double mutant of mice affected by staggerer and reeler conditions. I. The premature disappearance of the external granular layer and ensuing cerebellar disorganization. J. Neuropathol. Exp. Neurol. 36: 413–426.PubMedCrossRefGoogle Scholar
  199. Yoon, C. H. 1977b. Fine structure of the cerebellum of “staggerer-reeler,” a double mutant of mice affected by staggerer and reeler conditions. II. Purkinje cell anomalies. J. Neuropathol. Exp. Neurol 36: 427–439.PubMedCrossRefGoogle Scholar
  200. Yoon, C. H. 1977c. Fine structure of the cerebellum of “staggerer-reeler,” a double mutant of mice affected by staggerer and reeler conditions. III. Bergmann fiber anomalies. J. Neuropathol. Exp. Neurol 36: 440–452.CrossRefGoogle Scholar
  201. Zwann, J., and Williams, R. M. 1968. Cataracts and abnormal proliferation of the lens epithelium in mice carrying the Cat Frgene. Exp. Eye Res 8: 161–167.CrossRefGoogle Scholar
  202. Zwilling, E. 1941. The determination of the otic vesicle in Rana pipiens. J. Exp. Zool. 86: 333–342.CrossRefGoogle Scholar

Copyright information

© Plenum Press, New York 1985

Authors and Affiliations

  • Doris Burda Wilson
    • 1
  1. 1.Division of Anatomy, Department of Surgery, School of MedicineUniversity of California at San DiegoCaliforniaUSA

Personalised recommendations