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Gene Review

Pmp22  -  peripheral myelin protein 22

Rattus norvegicus

Synonyms: Cd25, PMP-22, Peripheral myelin protein 22, Pmp-22, Protein CD25, ...
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Disease relevance of Pmp22


High impact information on Pmp22

  • Migratory effector cells downregulate activation markers (CD25, OX-40) but upregulate several chemokine receptors and adsorb MHC class II on their membranes [5].
  • CD25-derived peptides function as ergotopes that can be recognized by the antiergotypic T cells [6].
  • Thus, CD25 DNA vaccination may induce protection from autoimmunity by inducing a cytokine shift in both the antiergotypic response and the response to the antigens targeted in the disease [6].
  • Membrane insertion of PMP 22 was demonstrated by protease treatment of peroxisomes in the absence and presence of detergent [7].
  • Characterization of a novel peripheral nervous system myelin protein (PMP-22/SR13) [8].

Biological context of Pmp22


Anatomical context of Pmp22


Associations of Pmp22 with chemical compounds

  • The synthesis of glycoprotein Po and peripheral myelin protein 22 in sciatic nerve of male rats is modulated by testosterone metabolites [15].
  • This concept is based on the findings that: (a) only 3alpha-diol, which is able to interact with GABAA receptor, is effective in stimulating the synthesis of PMP22 in the sciatic nerve of castrated male rats, and (b) flutamide treatment is ineffective in decreasing the protein levels in intact male rats [15].
  • Exogenous expression of PASII/PMP22 in C6 cells significantly inhibited BrdU incorporation to the cells [1].
  • Approximately 35% of total in vitro translated PMP 22 became protease resistant after a 1-h incubation at 26 degrees C. Import was dependent on time and temperature, did not require ATP or GTP and was not inhibited by N-ethylmaleimide treatment of neither the soluble components of the translation mixture nor of the isolated peroxisomes [7].
  • Role of the peripheral myelin protein 22 N-linked glycan in oligomer stability [16].

Regulatory relationships of Pmp22


Other interactions of Pmp22

  • For this reason, we currently do not have any functional data supporting that PASII/PMP22 is the member of claudin superfamily [1].
  • On the basis of the observations obtained in the Schwann cells cultures, we suggest that the stimulatory effect of neuroactive steroids on Po is acting through PR, while that on PMP22 needs the GABA(A) receptor [17].
  • The subcellular localization of PMP22 in cultured brain endothelia was confirmed by internalization with ZO-1 after EGTA-induced disruption of cell junctions [18].
  • Prior to the maturation of the BNB and BBB and detection of the tight junction protein occludin, PMP22 is present at ZO-1 positive endothelial junctions of the sciatic nerve and brain cortex [18].
  • The resulting SpL201 cell line expresses p75 and nestin, two proteins expressed by neural crest-derived cells, as well as peripheral myelin protein 22, protein zero, and Oct-6 as markers of the Schwann cell lineage [19].

Analytical, diagnostic and therapeutic context of Pmp22


  1. Functional analysis for peripheral myelin protein PASII/PMP22: is it a member of claudin superfamily? Takeda, Y., Notsu, T., Kitamura, K., Uyemura, K. Neurochem. Res. (2001) [Pubmed]
  2. An 8.5-kb segment of the PMP22 promoter responds to loss of axon signals during Wallerian degeneration, but does not respond to specific axonal signals during nerve regeneration. Orfali, W., Nicholson, R.N., Guiot, M.C., Peterson, A.C., Snipes, G.J. J. Neurosci. Res. (2005) [Pubmed]
  3. Proliferation of Schwann cells and regulation of cyclin D1 expression in an animal model of Charcot-Marie-Tooth disease type 1A. Atanasoski, S., Scherer, S.S., Nave, K.A., Suter, U. J. Neurosci. Res. (2002) [Pubmed]
  4. Studies on the effects of altered PMP22 expression during myelination in vitro. D'Urso, D., Schmalenbach, C., Zoidl, G., Prior, R., Müller, H.W. J. Neurosci. Res. (1997) [Pubmed]
  5. Migratory activity and functional changes of green fluorescent effector cells before and during experimental autoimmune encephalomyelitis. Flügel, A., Berkowicz, T., Ritter, T., Labeur, M., Jenne, D.E., Li, Z., Ellwart, J.W., Willem, M., Lassmann, H., Wekerle, H. Immunity (2001) [Pubmed]
  6. DNA vaccination with CD25 protects rats from adjuvant arthritis and induces an antiergotypic response. Mimran, A., Mor, F., Carmi, P., Quintana, F.J., Rotter, V., Cohen, I.R. J. Clin. Invest. (2004) [Pubmed]
  7. In vitro insertion of the 22-kD peroxisomal membrane protein into isolated rat liver peroxisomes. Diestelkötter, P., Just, W.W. J. Cell Biol. (1993) [Pubmed]
  8. Characterization of a novel peripheral nervous system myelin protein (PMP-22/SR13). Snipes, G.J., Suter, U., Welcher, A.A., Shooter, E.M. J. Cell Biol. (1992) [Pubmed]
  9. Axon-regulated expression of a Schwann cell transcript that is homologous to a 'growth arrest-specific' gene. Spreyer, P., Kuhn, G., Hanemann, C.O., Gillen, C., Schaal, H., Kuhn, R., Lemke, G., Müller, H.W. EMBO J. (1991) [Pubmed]
  10. Peripheral myelin protein 22 is a constituent of intercellular junctions in epithelia. Notterpek, L., Roux, K.J., Amici, S.A., Yazdanpour, A., Rahner, C., Fletcher, B.S. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
  11. Regional localization of rat peripheral myelin protein 22 (Pmp22) gene to chromosome 10q22 by FISH. Liehr, T., Rautenstrauss, B. Mamm. Genome (1995) [Pubmed]
  12. P0 and PMP22 mark a multipotent neural crest-derived cell type that displays community effects in response to TGF-beta family factors. Hagedorn, L., Suter, U., Sommer, L. Development (1999) [Pubmed]
  13. Sex-dimorphic effects of progesterone and its reduced metabolites on gene expression of myelin proteins by rat Schwann cells. Magnaghi, V., Veiga, S., Ballabio, M., Gonzalez, L.C., Garcia-Segura, L.M., Melcangi, R.C. J. Peripher. Nerv. Syst. (2006) [Pubmed]
  14. Temporal expression pattern of peripheral myelin protein 22 during in vivo and in vitro myelination. Notterpek, L., Snipes, G.J., Shooter, E.M. Glia (1999) [Pubmed]
  15. The synthesis of glycoprotein Po and peripheral myelin protein 22 in sciatic nerve of male rats is modulated by testosterone metabolites. Magnaghi, V., Ballabio, M., Gonzalez, L.C., Leonelli, E., Motta, M., Melcangi, R.C. Brain Res. Mol. Brain Res. (2004) [Pubmed]
  16. Role of the peripheral myelin protein 22 N-linked glycan in oligomer stability. Ryan, M.C., Notterpek, L., Tobler, A.R., Liu, N., Shooter, E.M. J. Neurochem. (2000) [Pubmed]
  17. Neuroactive steroids and peripheral myelin proteins. Magnaghi, V., Cavarretta, I., Galbiati, M., Martini, L., Melcangi, R.C. Brain Res. Brain Res. Rev. (2001) [Pubmed]
  18. The temporospatial expression of peripheral myelin protein 22 at the developing blood-nerve and blood-brain barriers. Roux, K.J., Amici, S.A., Notterpek, L. J. Comp. Neurol. (2004) [Pubmed]
  19. SpL201: a conditionally immortalized Schwann cell precursor line that generates myelin. Lobsiger, C.S., Smith, P.M., Buchstaller, J., Schweitzer, B., Franklin, R.J., Suter, U., Taylor, V. Glia (2001) [Pubmed]
  20. Coexpression of PMP22 gene with MBP and P0 during de novo myelination and nerve repair. Kuhn, G., Lie, A., Wilms, S., Müller, H.W. Glia (1993) [Pubmed]
  21. Progesterone derivatives are able to influence peripheral myelin protein 22 and P0 gene expression: possible mechanisms of action. Melcangi, R.C., Magnaghi, V., Cavarretta, I., Zucchi, I., Bovolin, P., D'Urso, D., Martini, L. J. Neurosci. Res. (1999) [Pubmed]
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