Summary
A new disorder of central myelination was found in black mutant hamsters showing trembling. The condition was inherited in an autosomal recessive mode. Histologically, a marked myelin deficiency was distributed throughout the central nervous system (CNS), and it was closely related to clinical manifestations observed in the mutants Ultrastructurally, abnormally thin myelin sheaths were observed in all axons with different diameters, and most axons below 1 μm in diameter were unmyelinated in the white matter of the spinal cord. There was no overt evidence of demyelination, but some myelin sheaths showed an aberrant configuration. The numbers of glial cells were not reduced, and there were no striking morphological abnormalities in them. It was suggested that there may be a dysfunction of oligodendrocytes resulting in retarded or defective myelination. This trembling hamsters may be a suitable animal model for the study of normal and abnormal myelinogenesis.
Similar content being viewed by others
References
Baumann NA, Harpin ML, Bourré JM (1970) Long chain fatty acid formation: Key step in myelination studies in mutant mice. Nature 227:960–961
Blakemore WF, Harding JDJ (1974) Ultrastructural observations on the spinal cords of piglets affected with congenital tremor type A IV. Res Vet Sci 17:248–255
Blakemore WF, Harding JDJ, Done JT (1974) Ultrastructural observations on the spinal cord of a Landrace pig with congenital tremor type A III. Res Vet Sci 17:174–178
Duchen IW, Eicher EM, Jacobs JM, Scaravilli F, Teixeira F (1980) Hereditary leucodystrophy in the mouse: The new mutant twitcher. Brain 103:695–710
Foulkes JA (1974) Myelin and dysmyelination in domestic animals. Vet Bull 8:441–450
Friedrich VL (1974) The myelin deficit in quaking mice. Brain Res 82:168–173
Griffiths IR, Duncan ID McCulloch M (1980) Shaking pups: A disorder of central myelination in the spaniel dog. II. Ultrastructural observations on the white matter of the cervical spinal cord. J Neurocytol 10:847–858
Hogan HL (1977) Animal models of genetic disorders of myelin. In: Morell P (ed) Myelin. Plenum Press, New York, pp 489–520
Ludwin SK (1978) Central nervous system demyelination and remyelination in the mouse: An ultrastructural study of cuprizone toxicity. Lab Invest 39:597–612
Matthieu JM, Widmer S, Herschkowitz N (1973) Jimpy, an anomaly myelin maturation. Biochemical study of myelination phases. Brain Res 55:403–412
Meier C, Bischoff A (1974) Dysmyelination in “jimpy” mouse. Electron-microscopic study. J Neuropathol Exp Neurol 33:343–353
Meier C, 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
Mikoshiba K (1984) Leucodystrophy in mice. In: Kyogoku K (ed) Experimental animal models of intractable diseases. Soft Science, Tokyo, pp 94–102
Patterson DSP, Done JT (1977) Neurochemistry as a diagnostic aid in the congenital tremor syndrome of piglets. Br Vet J 133:111–119
Samorajski T, Friede RL, Reimer PR (1970) Hypomyelination in the quaking mouse. J Neuropathol Exp Neurol 24:507–523
Skoff RP (1976) Myelin deficit in the jimpy mouse may be due to cellular abnormalities in astroglia. Nature 264:560–562
Vandevelde M, Braund KG, Luttgen PJ, Higgins RJ (1981) Dysmyelination in Chow Chow dogs: Further studies in older dogs. Acta Neuropathol (Berl) 55:81–87
Watanabe I, Bingle G (1972) Dysmyelination in “quaking” mouse. Electron-microscopic study. J Neuropathol Exp Neurol 31:352–369
Wiśniewski H, Raine CS (1971) An ultrastructural study of experimental demyelination and remyelination. V. Central and perpheral nervous system lesions caused by diphtheria toxin. Lab Invest 25:73–80
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Nunoya, T., Tajima, M., Mizutani, M. et al. A new mutant strain of syrian hamster with myelin deficiency. Acta Neuropathol 65, 305–312 (1985). https://doi.org/10.1007/BF00687013
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00687013