Document Detail


Targeted inactivation of a developmentally regulated neural plectin isoform (plectin 1c) in mice leads to reduced motor nerve conduction velocity.
MedLine Citation:
PMID:  19625254     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Cytolinker proteins stabilize cells mechanically, regulate cytoskeleton dynamics, and provide scaffolds for signaling molecules. For plectin, the prototype of these proteins, an unusual diversity of isoforms has been reported, which show distinct expression patterns, subcellular localizations, and functions. Plectin has been shown to have important functions in skin and muscle, but little is known about its role in neural cells. To address this issue, we generated two knock-out mouse lines, one which was selectively lacking plectin 1c (P1c), the major isoform expressed in neural cells, and another in which plectin was conditionally deleted in neuronal precursor cells. Using isoform-specific antibodies, we found P1c to be expressed late in development and to associate with postsynaptic dendrites of central nervous system neurons, motorneurons of spinal cord, sciatic nerve axons, and Schwann cells. Motor nerve conduction velocity was found significantly reduced in sciatic nerve from P1c-deficient as well as from conditional knock-out mice. This defect was traceable to an increased number of motor nerve fibers with small cross-sectional areas; the thicknesses of axons and of myelin sheaths were unaffected. This is the first report demonstrating an important role of plectin in a major nerve function.
Authors:
Peter Fuchs; Michael Zörer; Siegfried Reipert; Günther A Rezniczek; Friedrich Propst; Gernot Walko; Irmgard Fischer; Jan Bauer; Michael W Leschnik; Bernhard Lüscher; Johann G Thalhammer; Hans Lassmann; Gerhard Wiche
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2009-07-22
Journal Detail:
Title:  The Journal of biological chemistry     Volume:  284     ISSN:  1083-351X     ISO Abbreviation:  J. Biol. Chem.     Publication Date:  2009 Sep 
Date Detail:
Created Date:  2009-09-21     Completed Date:  2009-11-06     Revised Date:  2011-10-27    
Medline Journal Info:
Nlm Unique ID:  2985121R     Medline TA:  J Biol Chem     Country:  United States    
Other Details:
Languages:  eng     Pagination:  26502-9     Citation Subset:  IM    
Affiliation:
Department of Biochemistry and Cell Biology, Max F Perutz Laboratories, University of Vienna, 1030 Vienna, Austria.
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MeSH Terms
Descriptor/Qualifier:
Animals
Central Nervous System / metabolism
Female
Gene Targeting / methods*
Genotype
Immunoblotting
Immunohistochemistry
Male
Mice
Mice, Inbred C57BL
Mice, Knockout
Microscopy, Electron
Microscopy, Fluorescence
Motor Neurons / physiology*
Neural Conduction / physiology*
Plectin / genetics,  metabolism*
Protein Isoforms / genetics,  metabolism
Ranvier's Nodes / ultrastructure
Sciatic Nerve / metabolism,  physiology
Spinal Cord / metabolism
Spinal Nerve Roots / ultrastructure
Grant Support
ID/Acronym/Agency:
P 17862-B09//Austrian Science Fund FWF; P 20744-B11//Austrian Science Fund FWF
Chemical
Reg. No./Substance:
0/Plec protein, mouse; 0/Plectin; 0/Protein Isoforms
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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