The absence of myelin P0 protein produces a novel molecular phenotype in Schwann cells

Daniela Maria Menichella*, Wenbo Xu, Huiyuan Jiang, Jasloveleen Sohi, Jean Michael Vallat, Pierluigi Baron, John Kamholz, Michael Shy

*Corresponding author for this work

Research output: Contribution to journalArticle

4 Scopus citations

Abstract

In order to better understand the pathogenesis of demyelination in P0 knockout (P0 -/-) mice, we analyzed the myelin gene expression and the localization of myelin proteins in P0 null mouse sciatic nerve. We have demonstrated that the severe demyelinating neuropathy of P0-knockout mouse is associated with changes in the program of myelin gene expression. Some changes in myelin gene expression occur early, others occur during adulthood. We also provide evidence that the absence of P0 is associated with changes in the localization of specific paranodal proteins in the peripheral nerve. These data suggest that P0 plays an important role, either directly or indirectly, in the program of Schwann cell gene expression and in the specific distribution of peripheral myelin proteins. Furthermore, myelin gene dysregulation and improper localization of paranodal proteins may account, in part, for the pathogenesis of demyelination in P0-knockout mice, as well as in human demyelinating peripheral neuropathy associated with mutations in the P0 gene.

Original languageEnglish (US)
Pages (from-to)281-293
Number of pages13
JournalAnnals of the New York Academy of Sciences
Volume883
DOIs
StatePublished - Jan 1 1999

ASJC Scopus subject areas

  • Neuroscience(all)
  • Biochemistry, Genetics and Molecular Biology(all)
  • History and Philosophy of Science

Fingerprint Dive into the research topics of 'The absence of myelin P<sub>0</sub> protein produces a novel molecular phenotype in Schwann cells'. Together they form a unique fingerprint.

  • Cite this