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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

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

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