Aberrant splicing of a mouse disabled homolog, mdab1, in the scrambler mouse

Marcus L. Ware, Jeremy W. Fox, Jorge L. González, Nicole M. Davis, Catherine Lambert De Rouvroit, Christopher J. Russo, Streamson C. Chua, André M. Goffinet, Christopher A. Walsh

Research output: Contribution to journalArticle

221 Scopus citations

Abstract

Although accurate long-distance neuronal migration is a cardinal feature of cerebral cortical development, little is known about control of this migration. The scrambler (scm) mouse shows abnormal cortical lamination that is indistinguishable from reeler. Genetic and physical mapping of scm identified yeast artificial chromosomes containing an exon of mdab1, a homolog of Drosophila disabled, which encodes a phospho-protein that binds nonreceptor tyrosine kinases. mdab1 transcripts showed abnormal splicing in sero homozygotes, with 1.5 kb of intracisternal A particle retrotransposon sequence inserted into the mdab1 coding region in antisense orientation, producing a mutated and truncated predicted protein. Therefore, mdab1 is most likely the scm gene, thus implicating nonreceptor tyrosine kinases in neuronal migration and lamination in developing cerebral cortex.

Original languageEnglish (US)
Pages (from-to)239-249
Number of pages11
JournalNeuron
Volume19
Issue number2
DOIs
StatePublished - Jul 1997
Externally publishedYes

ASJC Scopus subject areas

  • Neuroscience(all)

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