Abnormal nerve conduction studies in mice expressing a mutant form of the POU transcription factor SCIP

Phyllis L. Bieri, Joseph C. Arezzo, David E. Weinstein

Research output: Contribution to journalArticle

6 Scopus citations

Abstract

We have previously described transgenic mice that harbor a dominant- negative antagonist of the POU protein SCIP (termed ΔSCIP). Native SCIP is expressed in promyelinating Schwann cells, where it repress expression of the myelin structural genes. The ΔSCIP mice display morphologic and behavioral abnormalities, including decreased axonal diameter, increased myelin thickness, developmentally early myelination, and clinical features of neuropathy. To assess the neurophysiologic correlates of these abnormalities, a series of electrophysiologic tests was performed. Despite having smaller diameter axons, mice expressing the ΔSCIP transgene had similar maximum condition velocities in caudal, sural, and tibial nerves compared to wild- type controls. Therefore, conduction in ΔSCIP animals was faster than predicted by axon diameter alone. Compound amplitude responses were 38% higher in the ΔSCIP caudal nerve. ΔSCIP tibial F-wave responses showed less difference between minimum and maximum latencies than controls, suggesting less variance between fastest and slowest conducting fibers. These data further characterize the functional components of the ΔSCIP phenotype. In addition, these studies address the physiologic sequelae of altering the g- ratio in the absence of demyelination or axonal degeneration.

Original languageEnglish (US)
Pages (from-to)821-828
Number of pages8
JournalJournal of Neuroscience Research
Volume50
Issue number5
DOIs
StatePublished - Dec 1 1997

Keywords

  • Axon
  • Myelin
  • Nerve conduction velocity
  • SCIP
  • Transgenic mouse

ASJC Scopus subject areas

  • Cellular and Molecular Neuroscience

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