Document Detail


Studies on the effects of altered PMP22 expression during myelination in vitro.
MedLine Citation:
PMID:  9086179     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
D D'Urso; C Schmalenbach; G Zoidl; R Prior; H W Müller
Related Documents :
3120179 - Accurate 5' splice-site selection in mouse kappa immunoglobulin light chain premessenge...
19176719 - Alternative splicing studies of the reactive oxygen species gene network in populus rev...
11342109 - Human ca2+/calmodulin-dependent phosphodiesterase pde1a: novel splice variants, their s...
20856809 - Fine-mapping resolves eae23 into two qtls and implicates zeb1 as a candidate gene regul...
15020709 - Bioinformatical assay of human gene morbidity.
11598999 - Altered gene expression in schwann cells of connexin32 knockout animals.
18278469 - The expression and bioinformatic analysis of a novel gene c20orf14 associated with lymp...
17299249 - Circadian rhythms in the cns and peripheral clock disorders: role of the biological clo...
11278649 - Inhibition of interleukin-4- and cd40-induced ige germline gene promoter activity by 2'...
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    
Affiliation:
Department of Neurology, Heinrich-Heine-University, Düsseldorf, Germany.
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Descriptor/Qualifier:
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:
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


Previous Document:  Protein from chromaffin granules promotes survival of mesencephalic dopaminergic neurons by an EGF-r...
Next Document:  Pharmacological characterisation of voltage-sensitive calcium channels and neurotransmitter release ...