| First Author | Xu W | Year | 2000 |
| Journal | J Neurosci Res | Volume | 60 |
| Issue | 6 | Pages | 714-24 |
| PubMed ID | 10861783 | Mgi Jnum | J:113170 |
| Mgi Id | MGI:3664698 | Doi | 10.1002/1097-4547(20000615)60:6<714::AID-JNR3>3.0.CO;2-1 |
| Citation | Xu W, et al. (2000) Absence of P0 leads to the dysregulation of myelin gene expression and myelin morphogenesis. J Neurosci Res 60(6):714-24 |
| abstractText | 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 (qk(v)), 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. |
