Extraocular muscle is spared upon complete laminin alpha2 chain deficiency: Comparative expression of laminin and integrin isoforms.
Författare
Summary, in English
Mutations in the gene encoding laminin (LM) alpha 2 chain cause congenital muscular dystrophy. Here, we show that extraocular muscle (EOM) is spared upon complete LM alpha 2 chain absence. The major LM chains in limb muscle basement membranes are alpha 2, beta 1, beta 2 and gamma 1 whereas alpha 2, alpha 4, beta 1, beta 2 and gamma 1 chains are expressed in EOM. Expression of LMoA chain mRNA is further increased in LM alpha 2 chain deficient EOM. Mainly integrin alpha 7X1 subunit, which binds to laminin-411, is expressed in EOM and in contrast to dystrophic limb muscle, sustained integrin OB expression is seen in LM alpha 2 chain deficient EOM. We propose that LM alpha 4 chain, possibly by binding to integrin alpha 7BX1 beta 1D, protects EOM in LM alpha 2 chain deficient muscular dystrophy. (c) 2006 Elsevier B.V./Intemational Society of Matrix Biology. All rights reserved.
Avdelning/ar
Publiceringsår
2006
Språk
Engelska
Sidor
382-385
Publikation/Tidskrift/Serie
Matrix Biology
Volym
25
Issue
May 16
Länkar
Dokumenttyp
Artikel i tidskrift
Förlag
Elsevier
Ämne
- Cell and Molecular Biology
Nyckelord
- laminin
- muscle
- muscular dystrophy
- integrin
- extracellular matrix
Status
Published
Forskningsgrupp
- Muscle Biology
ISBN/ISSN/Övrigt
- ISSN: 1569-1802