Myelin deficient mice: Expression of myelin basic protein and generation of mice with varying levels of myelin

Brian Popko*, Carmie Puckett, Eric Lai, H. David Shine, Carol Readhead, Naoki Takahashi, Stephen W. Hunt, Richard L. Sidman, Leroy Hood

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

160 Scopus citations

Abstract

Mice homozygous for the mutation myelin deficient (mld), an allele of shiverer, exhibit decreased CNS myelination, tremors, and convulsions of progressively increasing severity leading to an early death. In this report we demonstrate in mid mice that the gene encoding myelin basic protein (MBP) is expressed at decreased levels and on an abnormal temporal schedule relative to the wild-type gene. Southern blot analyses, field-inversion gel electrophoresis studies, and analyses of mld MBP cosmid clones indicate that there are multiple linked copies of the MBP gene in mld mice. We have introduced an MBP transgene into mld mice and found that myelination increases and tremors and convulsions decrease. Mld and shiverer mice with zero, one, or two copies of the MBP transgene express distinct levels of MBP mRNA and myelin. The availability of a range of mice expressing graded levels of myelin should facilitate quantitative analysis of the roles of MBP in the myelination process and of myelin in nerve function.

Original languageEnglish (US)
Pages (from-to)713-721
Number of pages9
JournalCell
Volume48
Issue number4
DOIs
StatePublished - Feb 27 1987

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

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