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.
journal_name
FEBS Lettjournal_title
FEBS lettersauthors
Gawlik KI,Mayer U,Blomberg K,Sonnenberg A,Ekblom P,Durbeej Mdoi
10.1016/j.febslet.2006.02.027keywords:
subject
Has Abstractpub_date
2006-03-20 00:00:00pages
1759-65issue
7eissn
0014-5793issn
1873-3468pii
S0014-5793(06)00225-0journal_volume
580pub_type
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