Journal article
The absence of myelin P0 protein produces a novel molecular phenotype in Schwann cells
Annals of the New York Academy of Sciences, Vol.883, pp.281-293
09/14/1999
DOI: 10.1111/j.1749-6632.1999.tb08590.x
PMID: 10586253
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.
Details
- Title: Subtitle
- The absence of myelin P0 protein produces a novel molecular phenotype in Schwann cells
- Creators
- Daniela maria Menichella - Institute of Neurology, University of Milan, IRCCS Ospedale Maggiore Policlinico, ItalyWenbo XuHuiyuan JiangJasloveleen SohiJean‐Michael VallatPierluigi BaronJohn KamholzMichael Shy
- Resource Type
- Journal article
- Publication Details
- Annals of the New York Academy of Sciences, Vol.883, pp.281-293
- DOI
- 10.1111/j.1749-6632.1999.tb08590.x
- PMID
- 10586253
- NLM abbreviation
- Ann N Y Acad Sci
- ISSN
- 0077-8923
- eISSN
- 1749-6632
- Publisher
- Blackwell Publishing Ltd; United States
- Language
- English
- Date published
- 09/14/1999
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Psychiatry; Stead Family Department of Pediatrics; Iowa Neuroscience Institute
- Record Identifier
- 9984020862002771
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