Journal article
Absence of P0 leads to the dysregulation of myelin gene expression and myelin morphogenesis
Journal of neuroscience research, Vol.60(6), pp.714-724
06/15/2000
DOI: 10.1002/1097-4547(20000615)60:6<714::AID-JNR3>3.0.CO;2-1
PMID: 10861783
Abstract
P0, the major peripheral nervous system (PNS) myelin protein, is a member of the immunoglobulin supergene family of membrane proteins and can mediate homotypic adhesion. P0 is an essential structural component of PNS myelin; mice in which P0 expression has been eliminated by homologous recombination (P0−/−) develop a severe dysmyelinating neuropathy with predominantly uncompacted myelin. Although P0 is thought to play a role in myelin compaction by promoting adhesion between adjacent extracellular myelin wraps, as an adhesion molecule it could also have a regulatory function. Consistent with this hypothesis, Schwann cells in adult P0−/− mice display a novel molecular phenotype: PMP22 expression is down‐regulated, MAG and PLP expression are up‐regulated, and MBP expression is unchanged. As in quaking viable mutant mice (qkv), which have uncompacted myelin morphologically similar to that found in P0−/− mice, neither the qKI‐6 or qKI‐7 proteins are expressed in P0−/− peripheral nerve. In addition to these changes in gene expression in the P0 knockout, PLP/DM‐20 accumulates in the endoplasmic reticulum of P0−/− Schwann cells, whereas MAG accumulates in redundant loops of uncompacted myelin, not at nodes of Ranvier or Schmidt‐Lantermann incisures. Taken together, these results demonstrate that P0 is involved, either directly or indirectly, in the regulation of both myelin gene expression and myelin morphogenesis. J. Neurosci. Res. 60:714–724, 2000. © 2000 Wiley‐Liss, Inc.
Details
- Title: Subtitle
- Absence of P0 leads to the dysregulation of myelin gene expression and myelin morphogenesis
- Creators
- Wenbo XuDaniela ManichellaHuiyuan JiangJean‐Michel VallatJack LilienPierluigi BaronGuglielmo ScarlatoJohn KamholzMichael E Shy
- Resource Type
- Journal article
- Publication Details
- Journal of neuroscience research, Vol.60(6), pp.714-724
- Publisher
- John Wiley & Sons, Inc; New York
- DOI
- 10.1002/1097-4547(20000615)60:6<714::AID-JNR3>3.0.CO;2-1
- PMID
- 10861783
- ISSN
- 0360-4012
- eISSN
- 1097-4547
- Number of pages
- 11
- Language
- English
- Date published
- 06/15/2000
- Academic Unit
- Neurology; Molecular Physiology and Biophysics; Psychiatry; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Biology
- Record Identifier
- 9984020894302771
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