Laminin alpha1 chain mediated reduction of laminin alpha2 chain deficient muscular dystrophy involves integrin alpha7beta1 and dystroglycan

FEBS Letters
Kinga I GawlikMadeleine Durbeej

Abstract

Transgenically introduced laminin (LN) alpha1 chain prevents muscular dystrophy in LNalpha2 chain deficient mice. We now report increased integrin alpha7Bbeta1D synthesis in dystrophic LNalpha2 chain deficient muscle. Yet, immunofluorescence demonstrated a reduced expression of integrin alpha7B subunit at the sarcolemma. Transgenic expression of LNalpha1 chain reconstituted integrin alpha7B at the sarcolemma. Expression of alpha- and beta-dystroglycan is enhanced in LNalpha2 chain deficient muscle and normalized by transgenic expression of LNalpha1 chain. We suggest that LNalpha1 chain in part ameliorates the development of LNalpha2 chain deficient muscular dystrophy by retaining the binding sites for integrin alpha7Bbeta1D and alpha-dystroglycan, respectively.

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Citations

Apr 12, 2007·Journal of Molecular Medicine : Official Organ of the Gesellschaft Deutscher Naturforscher Und Ärzte·Susanne SchéelePeter Ekblom
Nov 17, 2011·Cell and Tissue Research·Benjamin CharvetDominique Le Guellec
Jul 10, 2008·The Journal of Biological Chemistry·Mattias HägerMadeleine Durbeej
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Jul 30, 2011·Skeletal Muscle·Kinga I Gawlik, Madeleine Durbeej
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Mar 19, 2014·The American Journal of Pathology·Zandra KörnerMadeleine Durbeej
Jun 20, 2006·Matrix Biology : Journal of the International Society for Matrix Biology·Alexander NyströmMadeleine Durbeej
Nov 17, 2012·Cell Adhesion & Migration·Johan Holmberg, Madeleine Durbeej
Jan 8, 2014·The American Journal of Pathology·Kinga I GawlikMadeleine Durbeej
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Oct 4, 2016·Cellular and Molecular Life Sciences : CMLS·Yao Yao
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Aug 9, 2006·Human Molecular Genetics·Kinga I GawlikMadeleine Durbeej
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May 28, 2020·Frontiers in Molecular Neuroscience·Kinga I Gawlik, Madeleine Durbeej
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Apr 20, 2021·Molecular Therapy. Methods & Clinical Development·Davin Packer, Paul T Martin

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