Laminin alpha1 chain mediated reduction of laminin alpha2 chain deficient muscular dystrophy involves integrin alpha7beta1 and dystroglycan.
Författare
Summary, in English
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.
Publiceringsår
2006
Språk
Engelska
Sidor
1759-1765
Publikation/Tidskrift/Serie
FEBS Letters
Volym
580
Issue
7
Länkar
Dokumenttyp
Artikel i tidskrift
Förlag
Wiley-Blackwell
Ämne
- Biological Sciences
Nyckelord
- Integrin
- Dystroglycan
- Laminin
- Muscular dystrophy
Status
Published
Forskningsgrupp
- Muscle Biology
ISBN/ISSN/Övrigt
- ISSN: 1873-3468