AI Article Synopsis
- Transgenic expression of the laminin alpha1 chain prevents muscular dystrophy in mice lacking the laminin alpha2 chain.
- In these deficient mice, the synthesis of integrin alpha7Bbeta1D is increased, but the expression of the integrin alpha7B subunit at the muscle cell surface (sarcolemma) is reduced.
- Introducing laminin alpha1 chain restores the proper expression of integrin alpha7B and normalizes levels of alpha- and beta-dystroglycan, suggesting it helps maintain key binding sites that are crucial for muscle function.
Article 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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| http://dx.doi.org/10.1016/j.febslet.2006.02.027 | DOI Listing |
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