Expression of a myelin basic protein gene in transgenic shiverer mice: Correction of the dysmyelinating phenotype

Carol Readhead*, Brian Popko, Naoki Takahashi, H. David Shine, Raul A. Saavedra, Richard L. Sidman, Leroy Hood

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

278 Scopus citations

Abstract

Mice homozygous for the autosomal recessive mutation shiverer (shi) lack myelin basic protein (MBP) and exhibit a distinct behavioral pattern including tremors (shivering), convulsions, and early death. We have previously demonstrated that shiverer mice have a partial deletion in the gene encoding MBP. We now have introduced the wild-type MBP gene into the germ line of shiverer mice by microinjection into fertilized eggs. Transgenic shiverer mice homozygous for the introduced gene have MBP mRNA and protein levels that are approximately 25% of normal, and produce compacted myelin with major dense lines. Correct temporal and spatial expression of the MBP gene is achieved with a genomic MBP cosmid clone containing 4 kb of 5′ flanking sequence and 1 kb of 3′ flanking sequence. Moreover, the four different forms of MBP produced by alternative patterns of RNA splicing are present. These homozygous transgenic shiverer mice no longer shiver nor die prematurely.

Original languageEnglish (US)
Pages (from-to)703-712
Number of pages10
JournalCell
Volume48
Issue number4
DOIs
StatePublished - Feb 27 1987

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

Fingerprint Dive into the research topics of 'Expression of a myelin basic protein gene in transgenic shiverer mice: Correction of the dysmyelinating phenotype'. Together they form a unique fingerprint.

Cite this