Laminin α1 chain mediated reduction of laminin α2 chain deficient muscular dystrophy involves integrin α7β1 and dystroglycan
- 21 February 2006
- journal article
- Published by Wiley in FEBS Letters
- Vol. 580 (7) , 1759-1765
- https://doi.org/10.1016/j.febslet.2006.02.027
Abstract
Transgenically introduced laminin (LN) α1 chain prevents muscular dystrophy in LNα2 chain deficient mice. We now report increased integrin α7Bβ1D synthesis in dystrophic LNα2 chain deficient muscle. Yet, immunofluorescence demonstrated a reduced expression of integrin α7B subunit at the sarcolemma. Transgenic expression of LNα1 chain reconstituted integrin α7B at the sarcolemma. Expression of α‐ and β‐dystroglycan is enhanced in LNα2 chain deficient muscle and normalized by transgenic expression of LNα1 chain. We suggest that LNα1 chain in part ameliorates the development of LNα2 chain deficient muscular dystrophy by retaining the binding sites for integrin α7Bβ1D and α‐dystroglycan, respectively.Keywords
This publication has 29 references indexed in Scilit:
- A simplified laminin nomenclatureMatrix Biology, 2005
- Overexpression of mini‐agrin in skeletal muscle increases muscle integrity and regenerative capacity in laminin‐α2‐deficient miceThe FASEB Journal, 2005
- Dystroglycan: important player in skeletal muscle and beyondNeuromuscular Disorders, 2005
- Laminin 1 chain reduces muscular dystrophy in laminin 2 chain deficient miceHuman Molecular Genetics, 2004
- Defective integrin switch and matrix composition at alpha 7-deficient myotendinous junctions precede the onset of muscular dystrophy in miceHuman Molecular Genetics, 2003
- Post-translational disruption of dystroglycan–ligand interactions in congenital muscular dystrophiesNature, 2002
- Alternative Splice Variants of α7β1Integrin Selectively Recognize Different Laminin IsoformsJournal of Biological Chemistry, 2002
- An agrin minigene rescues dystrophic symptoms in a mouse model for congenital muscular dystrophyNature, 2001
- Spatial and temporal expression of the β1D integrin during mouse developmentDevelopmental Dynamics, 1997
- Primary structure of dystrophin-associated glycoproteins linking dystrophin to the extracellular matrixNature, 1992