Proceedings of The Japanese Association of Animal Models for Human Diseases
Online ISSN : 1884-4189
Print ISSN : 0911-2057
ISSN-L : 0911-2057
Successful Treatment with Forphenicinol, an Immunomodifier, to Prevent the Defective Myelination in Jimpy Mouse
Shigekatsu TSUJI
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1985 Volume 1 Pages 13-17

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Abstract
Jimpy mouse is a neurological mutant characterized by a defective myelination in the central nervous system and the spontaneous occurrence of severe intention tremor, unsteady gait and gen-eralized tonic-clonic seizures. The primary cause of the myelin deficiency has not yet been determined, although there have been numerous morphologic and biochemical studies.
In order to elucidate more clearly the disturbances of myelination in the central nervous system, we investigated the continuous administration of Forphenicinol, a small molecular weight im-munomodifier isolated from the culture of actinomycetes, to Jimpy mouse at early postnatal age to find the preventive effects of this substance on the development of clinical manifestation of the behavioral abnormalities and early death. The results obtained were summarized as follows:
1) Continuous administration of Forphenicinol at early postnatal age resulted in a striking recovery of myelin associated CNPase and CEHase activities and of total myelin concentration in the central nervous system of the Jimpy mouse.
2) A complete restration from clinical symptoms of intention tremor and convulsive seizures are appeared in about sixty percent members of Forphenicinol administrated Jimpy mice. The recovering mice also showed a marked prolongation of their life span.
3) Synthesis of galactocerebrosides, a specific lipid component of the myelin structure which is practically absent in the central nervous system of Jimpy mice, are regained to normal after administration of Forphenicinol.
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© Japanese Association for Laboratory Animal Science
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