| Studies on the effects of altered PMP22 expression during myelination in vitro. | |
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MedLine Citation:
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PMID: 9086179 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Severe inherited dysmyelinating diseases of the peripheral nervous system, the Charcot-Marie-Tooth type1A disease (CMT1A) and the hereditary neuropathy with liability to pressure palsies (HNPP) are associated with a large DNA duplication or deletion of a chromosomal region containing the peripheral myelin protein 22 (PMP22) gene. It has been suggested that a gene dosage effect involving PMP22 is responsible for the pathological phenotype. We investigated if altered PMP22 expression affects the onset of myelin formation and the ultrastructure of myelin. Rat Schwann cell cultures were stably infected with recombinant retrovirus vectors harboring the rat PMP22 cDNA in sense or antisense orientation. Schwann cells over- or underexpressing PMP22 were cocultured with purified DRG neurons under conditions that promote myelination. We examined PMP22 expression and localization in the myelin forming cultures by RT-PCR, immunohistochemistry and confocal microscopy, and we analyzed myelin ultrastructure by electron microscopy. Our results demonstrate that abnormal levels of PMP22 expression do not impair the early stages of myelination and membrane compaction and do not interfere with the expression of other myelin genes. Our observations further indicate that PMP22 is involved more in controlling myelin thickness and stability than in the events determining the initial steps of myelin formation. |
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Authors:
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D D'Urso; C Schmalenbach; G Zoidl; R Prior; H W Müller |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Journal of neuroscience research Volume: 48 ISSN: 0360-4012 ISO Abbreviation: J. Neurosci. Res. Publication Date: 1997 Apr |
Date Detail:
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Created Date: 1997-06-09 Completed Date: 1997-06-09 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 7600111 Medline TA: J Neurosci Res Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 31-42 Citation Subset: IM |
Affiliation:
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Department of Neurology, Heinrich-Heine-University, Düsseldorf, Germany. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Animals Animals, Newborn Cells, Cultured / chemistry, physiology, ultrastructure Ganglia, Spinal / cytology Gene Expression / physiology Gene Transfer Techniques In Situ Hybridization Microscopy, Confocal Microscopy, Electron Myelin Proteins / analysis, genetics* Myelin Sheath / chemistry, physiology*, ultrastructure Neurons / chemistry, cytology, physiology Polymerase Chain Reaction RNA, Messenger / analysis Rats Rats, Wistar Recombinant Proteins / genetics Retroviridae Schwann Cells / chemistry, cytology, physiology |
| Chemical | |
Reg. No./Substance:
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0/Myelin Proteins; 0/Pmp22 protein, rat; 0/RNA, Messenger; 0/Recombinant Proteins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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