Chimeric and molecular genetic analysis of myelin-deficiency (shiverer and mld) mutant mice

K. Mikoshiba, Hideyuki Okano, J. Aruga, T. A. Tamura, M. Miura, K. Ikenaka, T. Nakagawa

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Abstract

The shiverer and myelin-deficient (mld) mutants are two allelic mutations. Both are characterized by hypomyelination in the CNS and deficient expression of the MBP gene. Chimeric analysis of the pathogenesis of shiverer showed that shiverer mutation acts intrinsic to the oligodendrocyte, which is the only cell type expressing. The MBP gene in the CNS. Molecular genetic studies by several groups demonstrated that shiverer is a deletion mutation in the MBP gene. Consequently, no MBP is produced in the shiverer mutant. In mld, however, partial expression of the MBP gene was observed. Interestingly, MBP was expressed in a mosaic fashion in the CNS of mld mice. Molecular genetic studies revealed that the mld mutant has duplicated MBP genes in tandem on a distal part (E2-4) of chromosome 18q. The reduced expression was based on the level of mRNA. A large portion is inverted in the upstream copy, and an intact copy is located downstream. We showed in mld mutants that antisense RNA corresponding to the inverted segment is transcribed, and it forms RNA duplex, with the RNA transcribed from the normal gene located downstream.

Original languageEnglish
Pages (from-to)166-182
Number of pages17
JournalAnnals of the New York Academy of Sciences
Volume605
Publication statusPublished - 1990
Externally publishedYes

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Myelin Sheath
Molecular Biology
Genes
RNA
Gene Expression
Antisense RNA
Mutation
Chromosomes, Human, Pair 4
Sequence Deletion
Oligodendroglia
Chromosomes
Molecular Genetics
Mouse
Gene
Messenger RNA
Central Nervous System

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

Cite this

Mikoshiba, K., Okano, H., Aruga, J., Tamura, T. A., Miura, M., Ikenaka, K., & Nakagawa, T. (1990). Chimeric and molecular genetic analysis of myelin-deficiency (shiverer and mld) mutant mice. Annals of the New York Academy of Sciences, 605, 166-182.

Chimeric and molecular genetic analysis of myelin-deficiency (shiverer and mld) mutant mice. / Mikoshiba, K.; Okano, Hideyuki; Aruga, J.; Tamura, T. A.; Miura, M.; Ikenaka, K.; Nakagawa, T.

In: Annals of the New York Academy of Sciences, Vol. 605, 1990, p. 166-182.

Research output: Contribution to journalArticle

Mikoshiba, K, Okano, H, Aruga, J, Tamura, TA, Miura, M, Ikenaka, K & Nakagawa, T 1990, 'Chimeric and molecular genetic analysis of myelin-deficiency (shiverer and mld) mutant mice', Annals of the New York Academy of Sciences, vol. 605, pp. 166-182.
Mikoshiba, K. ; Okano, Hideyuki ; Aruga, J. ; Tamura, T. A. ; Miura, M. ; Ikenaka, K. ; Nakagawa, T. / Chimeric and molecular genetic analysis of myelin-deficiency (shiverer and mld) mutant mice. In: Annals of the New York Academy of Sciences. 1990 ; Vol. 605. pp. 166-182.
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