NDLI: Targeted inactivation of a developmentally regulated neural plectin isoform (plectin 1c) in mice leads to reduced motor nerve conduction velocity.

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Targeted inactivation of a developmentally regulated neural plectin isoform (plectin 1c) in mice leads to reduced motor nerve conduction velocity.

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Targeted inactivation of a developmentally regulated neural plectin isoform (plectin 1c) in mice leads to reduced motor nerve conduction velocity.

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Bauer, Jan Fuchs, Peter Walko, Gernot Propst, Friedrich Lüscher, Bernhard Fischer, Irmgard Rezniczek, Günther A. Zörer, Michael Leschnik, Michael W. Lassmann, Hans Wiche, Gerhard Reipert, Siegfried Thalhammer, Johann G.
Description	Author Affiliation: Fuchs P ( Department of Biochemistry and Cell Biology, Max F Perutz Laboratories, University of Vienna, 1030 Vienna, Austria.)
Abstract	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.
ISSN	00219258
e-ISSN	1083351X
Journal	Journal of Biological Chemistry
Issue Number	39
Volume Number	284
Language	English
Publisher	American Society for Biochemistry and Molecular Biology (United States)
Publisher Date	2009-09-25
Publisher Place	United States
Access Restriction	Open
Subject Keyword	Gene Targeting Motor Neurons Physiology Neural Conduction Plectin Metabolism Animals Central Nervous System Genotype Immunoblotting Immunohistochemistry Mice Mice, Inbred C57BL Mice, Knockout Microscopy, Electron Microscopy, Fluorescence Genetics Protein Isoforms Ranvier's Nodes Ultrastructure Sciatic Nerve Spinal Cord Spinal Nerve Roots Research Support, Non-U.S. Gov't Biochemistry Molecular Biology
Content Type	Text
Resource Type	Article
Subject	Cell Biology Biochemistry Molecular Biology

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