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Deletion of Integrin-Linked Kinase from Skeletal Muscles of Mice Resembles Muscular Dystrophy Due to {alpha}7 1-Integrin Deficiency.

Deletion of Integrin-Linked Kinase from Skeletal Muscles of Mice Resembles Muscular Dystrophy Due to {alpha}7 1-Integrin Deficiency. Research Abstract Details 

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  • Deletion of Integrin-Linked Kinase from Skeletal Muscles of Mice Resembles Muscular Dystrophy Due to {alpha}7 1-Integrin Deficiency. Abstract Text:

    ania l gheyaraAnia L Gheyara,ainara vallejo-illarramendiAinara Vallejo-Illarramendi,keling zangKeling Zang,lin meiLin Mei,rene st-arnaudRene St-Arnaud,shoukat dedharShoukat Dedhar,louis f reichardtLouis F Reichardt,ania l gheyaraAnia L Gheyara,ainara vallejo-illarramendiAinara Vallejo-Illarramendi,keling zangKeling Zang,lin meiLin Mei,rene st-arnaudRene St-Arnaud,shoukat dedharShoukat Dedhar,louis f reichardtLouis F Reichardt,ania l gheyaraAnia L Gheyara,ainara vallejo-illarramendiAinara Vallejo-Illarramendi,keling zangKeling Zang,lin meiLin Mei,rene st-arnaudRene St-Arnaud,shoukat dedharShoukat Dedhar,louis f reichardtLouis F Reichardt,

    Integrin-linked kinase (Ilk) is a serine/threonine kinase and an adaptor protein that links integrins to the actin cytoskeleton and to a number of signaling pathways involved in integrin action. We hypothesized that Ilk may act as an important effector of integrins in skeletal muscle, where these receptors provide a critical link between the sarcolemma and the extracellular matrix. Using the cre/lox system, we deleted Ilk from skeletal muscles of mice. The resulting mutants developed a progressive muscular dystrophy with multiple degenerating and regenerating muscle fibers, increased central nuclei, and endomysial fibrosis. These defects were widespread but were most severe near myofascial junctions where Ilk mutants showed displacement of focal adhesion-related proteins, including vinculin, paxillin, focal adhesion kinase, dystrophin, and the alpha7beta1D-integrin subunits. Distal ends of mutant muscle fibers appeared irregular, and there was restructuring of the actin cytoskeleton. These findings resemble those seen in humans and mice lacking the alpha7-integrin subunit and suggest that Ilk may act as a cytoplasmic effector of alpha7beta1-integrin in the pathogenesis of these deficiencies.

    Deletion of Integrin-Linked Kinase from Skeletal Muscles of Mice Resembles Muscular Dystrophy Due to {alpha}7 1-Integrin Deficiency. Publishing Authors By Initials

    al gheyaraAL Gheyara,a vallejo-illarramendiA Vallejo-Illarramendi,k zangK Zang,l meiL Mei,r st-arnaudR St-Arnaud,s dedharS Dedhar,lf reichardtLF Reichardt,al gheyaraAL Gheyara,a vallejo-illarramendiA Vallejo-Illarramendi,k zangK Zang,l meiL Mei,r st-arnaudR St-Arnaud,s dedharS Dedhar,lf reichardtLF Reichardt,al gheyaraAL Gheyara,a vallejo-illarramendiA Vallejo-Illarramendi,k zangK Zang,l meiL Mei,r st-arnaudR St-Arnaud,s dedharS Dedhar,lf reichardtLF Reichardt,

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    Deletion of Integrin-Linked Kinase from Skeletal Muscles of Mice Resembles Muscular Dystrophy Due to {alpha}7 1-Integrin Deficiency. Journal Published:

    PUBLICATION TYPE: Journal Article

    Journal: The American journal of pathology

    VOLUME: 171

    Page Numbers: 1966-77

    Journal Abbreviation: Am. J. Pathol.

    ISSN: 0002-9440

    DAY: 30

    MONTH: 11

    YEAR: 2007

    Deletion of Integrin-Linked Kinase from Skeletal Muscles of Mice Resembles Muscular Dystrophy Due to {alpha}7 1-Integrin Deficiency. Information

    Number of References:

    LANGUAGE: eng

    NlmUniqueID: 370502

    Deletion of Integrin-Linked Kinase from Skeletal Muscles of Mice Resembles Muscular Dystrophy Due to {alpha}7 1-Integrin Deficiency. Keywords Mesh Terms:

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    Chemical & Substance for Abstract: Deletion of Integrin-Linked Kinase from Skeletal Muscles of Mice Resembles Muscular Dystrophy Due to {alpha}7 1-Integrin Deficiency. Information

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    Grant and Affiliation Information for Deletion of Integrin-Linked Kinase from Skeletal Muscles of Mice Resembles Muscular Dystrophy Due to {alpha}7 1-Integrin Deficiency.

    AFFILIATION: Department of Physiology, University of California, San Francisco, San Francisco, California 94143. louis.Reichardt@ucsf.edu.

    Country: United States

    United States Research PublicationUnited States Research Publication

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    MEDLINETA: Am J Pathol

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    Deletion of Integrin-Linked Kinase from Skeletal Muscles of Mice Resembles Muscular Dystrophy Due to {alpha}7 1-Integrin Deficiency Related Publications

     

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