QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

HOME  |   SEARCH  |   ARCHIVE  |   SUBSCRIBE  |   CONTACT  |   HELP

This Article Full Text Full Text (PDF) Submit an eLetter Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in Web of Science Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Web of Science (35) Citing Articles via Google Scholar Google Scholar Articles by Niemann, S. Articles by Nave, K.-A. Search for Related Content PubMed PubMed Citation Articles by Niemann, S. Articles by Nave, K.-A. Pubmed/NCBI databases OMIM Compound via MeSH Substance via MeSH

 Previous Article  |  Next Article 

Uncoupling of Myelin Assembly and Schwann Cell Differentiation by Transgenic Overexpression of Peripheral Myelin Protein 22

Stephan Niemann¹, Michael W. Sereda¹, Ueli Suter², Ian R. Griffiths³, and Klaus-Armin Nave¹

¹ Zentrum für Molekulare Biologie (ZMBH), University of Heidelberg, D-69120 Heidelberg, Germany, ² Institute of Cell Biology, Department of Biology, Swiss Federal Institute of Technology, Eidgenössische Technische Hochschule-Hoenggerberg, CH-8093 Zürich, Switzerland, and ³ Applied Neurobiology Group, Department of Veterinary Clinical Studies, University of Glasgow, Glasgow G61 1QH, United Kingdom

We have generated previously transgenic rats that overexpress peripheral myelin protein 22 (PMP22) in Schwann cells. In the nerves of these animals, Schwann cells have segregated with axons to the normal 1:1 ratio but remain arrested at the promyelinating stage, apparently unable to elaborate myelin sheaths. We have examined gene expression of these dysmyelinating Schwann cells using semiquantitative reverse transcription-PCR and immunofluorescence analysis. Unexpectedly, Schwann cell differentiation appears to proceed normally at the molecular level when monitored by the expression of mRNAs encoding major structural proteins of myelin. Furthermore, an aberrant coexpression of early and late Schwann cell markers was observed. PMP22 itself acquires complex glycosylation, suggesting that trafficking of the myelin protein through the endoplasmic reticulum is not significantly impaired. We suggest that PMP22, when overexpressed, accumulates in a late Golgi-cell membrane compartment and uncouples myelin assembly from the underlying program of Schwann cell differentiation.

Key words: transgenic disease model; Charcot-Marie-Tooth neuropathy; myelin disease; axon-Schwann cell interaction; abnormal gene dosage; myelin protein function

---

Copyright © 2000 Society for Neuroscience  0270-6474/00/20114120-09$05.00/0

This article has been cited by other articles:

				L. Nobbio, L. Sturla, F. Fiorese, C. Usai, G. Basile, I. Moreschi, F. Benvenuto, E. Zocchi, A. De Flora, A. Schenone, et al. P2X7-mediated Increased Intracellular Calcium Causes Functional Derangement in Schwann Cells from Rats with CMT1A Neuropathy J. Biol. Chem., August 21, 2009; 284(34): 23146 - 23158. [Abstract] [Full Text] [PDF]

				R. Chies, L. Nobbio, P. Edomi, A. Schenone, C. Schneider, and C. Brancolini Alterations in the Arf6-regulated plasma membrane endosomal recycling pathway in cells overexpressing the tetraspan protein Gas3/PMP22 J. Cell Sci., March 15, 2003; 116(6): 987 - 999. [Abstract] [Full Text] [PDF]

				K. M. Dickson, J. J. M. Bergeron, I. Shames, J. Colby, D. T. Nguyen, E. Chevet, D. Y. Thomas, and G. J. Snipes Association of calnexin with mutant peripheral myelin protein-22 ex vivo: A basis for "gain-of-function" ER diseases PNAS, July 23, 2002; 99(15): 9852 - 9857. [Abstract] [Full Text] [PDF]

				S. Sancho, P. Young, and U. Suter Regulation of Schwann cell proliferation and apoptosis in PMP22-deficient mice and mouse models of Charcot-Marie-Tooth disease type 1A Brain, November 1, 2001; 124(11): 2177 - 2187. [Abstract] [Full Text] [PDF]

				J. Perea, A. Robertson, T. Tolmachova, J. Muddle, R. H. M. King, S. Ponsford, P. K. Thomas, and C. Huxley Induced myelination and demyelination in a conditional mouse model of Charcot-Marie-Tooth disease type 1A Hum. Mol. Genet., May 1, 2001; 10(10): 1007 - 1018. [Abstract] [Full Text] [PDF]

				S. Schneider, F. Bosse, D. D'Urso, H.-W. Muller, M. W. Sereda, K.-A. Nave, A. Niehaus, T. Kempf, M. Schnolzer, and J. Trotter The AN2 Protein Is a Novel Marker for the Schwann Cell Lineage Expressed by Immature and Nonmyelinating Schwann Cells J. Neurosci., February 1, 2001; 21(3): 920 - 933. [Abstract] [Full Text] [PDF]

Home  |   Search  |   Archive  |   Subscribe  |   Contact  |   Help