Document Detail

Studies on the effects of altered PMP22 expression during myelination in vitro.
MedLine Citation:
PMID:  9086179     Owner:  NLM     Status:  MEDLINE    
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.
D D'Urso; C Schmalenbach; G Zoidl; R Prior; H W Müller
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of neuroscience research     Volume:  48     ISSN:  0360-4012     ISO Abbreviation:  J. Neurosci. Res.     Publication Date:  1997 Apr 
Date Detail:
Created Date:  1997-06-09     Completed Date:  1997-06-09     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7600111     Medline TA:  J Neurosci Res     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  31-42     Citation Subset:  IM    
Department of Neurology, Heinrich-Heine-University, Düsseldorf, Germany.
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MeSH Terms
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, Wistar
Recombinant Proteins / genetics
Schwann Cells / chemistry,  cytology,  physiology
Reg. No./Substance:
0/Myelin Proteins; 0/Pmp22 protein, rat; 0/RNA, Messenger; 0/Recombinant Proteins

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