| Targeted inactivation of a developmentally regulated neural plectin isoform (plectin 1c) in mice leads to reduced motor nerve conduction velocity. | |
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MedLine Citation:
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PMID: 19625254 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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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:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2009-07-22 |
Journal Detail:
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Title: The Journal of biological chemistry Volume: 284 ISSN: 1083-351X ISO Abbreviation: J. Biol. Chem. Publication Date: 2009 Sep |
Date Detail:
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Created Date: 2009-09-21 Completed Date: 2009-11-06 Revised Date: 2011-10-27 |
Medline Journal Info:
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Nlm Unique ID: 2985121R Medline TA: J Biol Chem Country: United States |
Other Details:
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Languages: eng Pagination: 26502-9 Citation Subset: IM |
Affiliation:
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Department of Biochemistry and Cell Biology, Max F Perutz Laboratories, University of Vienna, 1030 Vienna, Austria. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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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:
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P 17862-B09//Austrian Science Fund FWF; P 20744-B11//Austrian Science Fund FWF |
| Chemical | |
Reg. No./Substance:
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0/Plec protein, mouse; 0/Plectin; 0/Protein Isoforms |
| Comments/Corrections | |
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