Document Detail


The claw paw mutation reveals a role for Lgi4 in peripheral nerve development.
MedLine Citation:
PMID:  16341215     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Peripheral nerve development results from multiple cellular interactions between axons, Schwann cells and the surrounding mesenchymal tissue. The delayed axonal sorting and hypomyelination throughout the peripheral nervous system of claw paw (clp) mutant mice suggest that the clp gene product is critical for these interactions. Here we identify the clp mutation as a 225-bp insertion in the Lgi4 gene. Lgi4 encodes a secreted and glycosylated leucine-rich repeat protein and is expressed in Schwann cells. The clp mutation affects Lgi4 mRNA splicing, resulting in a mutant protein that is retained in the cell. Additionally, siRNA-mediated downregulation of Lgi4 in wild-type neuron-Schwann cell cocultures inhibits myelination, whereas exogenous Lgi4 restores myelination in clp/clp cultures. Thus, the abnormalities observed in clp mice are attributable to the loss of Lgi4 function, and they identify Lgi4 as a new component of Schwann cell signaling pathway(s) that controls axon segregation and myelin formation.
Authors:
John R Bermingham; Harold Shearin; Jamie Pennington; Jill O'Moore; Martine Jaegle; Siska Driegen; Arend van Zon; Aysel Darbas; Ekim Ozkaynak; Elizabeth J Ryu; Jeffrey Milbrandt; Dies Meijer
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't     Date:  2005-12-11
Journal Detail:
Title:  Nature neuroscience     Volume:  9     ISSN:  1097-6256     ISO Abbreviation:  Nat. Neurosci.     Publication Date:  2006 Jan 
Date Detail:
Created Date:  2005-12-26     Completed Date:  2006-02-27     Revised Date:  2013-06-05    
Medline Journal Info:
Nlm Unique ID:  9809671     Medline TA:  Nat Neurosci     Country:  United States    
Other Details:
Languages:  eng     Pagination:  76-84     Citation Subset:  IM    
Affiliation:
McLaughlin Research Institute, 1520 23rd Street South, Great Falls, Montana 59405, USA. jrbjr@po.mri.montana.edu
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MeSH Terms
Descriptor/Qualifier:
Amino Acid Sequence
Animals
Axons / physiology
Base Sequence
Cloning, Molecular
Coculture Techniques
DNA Transposable Elements
DNA, Complementary / biosynthesis,  genetics
Down-Regulation / genetics,  physiology
Foot Deformities / genetics*
Gene Expression Regulation / genetics,  physiology
Genetic Complementation Test
Genotype
Immunohistochemistry
In Situ Hybridization
Lentivirus / genetics
Mice
Mice, Inbred C57BL
Mice, Knockout
Molecular Sequence Data
Mutation / physiology*
Myelin Sheath / physiology
Neurons, Afferent / physiology
Peripheral Nervous System / growth & development*,  physiology*
Phenotype
Proteins / genetics,  physiology*
RNA, Small Interfering / genetics
Rats
Reverse Transcriptase Polymerase Chain Reaction
Schwann Cells / physiology
Transfection
Grant Support
ID/Acronym/Agency:
NS049087/NS/NINDS NIH HHS; NS40745/NS/NINDS NIH HHS; NS40751/NS/NINDS NIH HHS; R01 NS040751/NS/NINDS NIH HHS
Chemical
Reg. No./Substance:
0/DNA Transposable Elements; 0/DNA, Complementary; 0/Lgi4 protein, mouse; 0/Proteins; 0/RNA, Small Interfering

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


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