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Extraocular muscle is spared upon complete laminin alpha2 chain deficiency: Comparative expression of laminin and integrin isoforms.

Nyström, Alexander LU ; Holmblad, Johanna; Pedrosa-Domellof, Fatima; Sasaki, Takako and Durbeej-Hjalt, Madeleine LU (2006) In Matrix Biology 25(May 16). p.382-385
Abstract
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... (More)
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. (Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
laminin, muscle, muscular dystrophy, integrin, extracellular matrix
in
Matrix Biology
volume
25
issue
May 16
pages
382 - 385
publisher
Elsevier
external identifiers
  • wos:000240595400008
  • scopus:33746949360
ISSN
1569-1802
DOI
10.1016/j.matbio.2006.05.001
language
English
LU publication?
yes
id
f0be988c-4b18-4ebb-95d7-8a83595d0dc3 (old id 158261)
alternative location
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16782317&dopt=Abstract
date added to LUP
2007-07-18 13:34:15
date last changed
2019-02-20 07:47:59
@article{f0be988c-4b18-4ebb-95d7-8a83595d0dc3,
  abstract     = {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.},
  author       = {Nyström, Alexander and Holmblad, Johanna and Pedrosa-Domellof, Fatima and Sasaki, Takako and Durbeej-Hjalt, Madeleine},
  issn         = {1569-1802},
  keyword      = {laminin,muscle,muscular dystrophy,integrin,extracellular matrix},
  language     = {eng},
  number       = {May 16},
  pages        = {382--385},
  publisher    = {Elsevier},
  series       = {Matrix Biology},
  title        = {Extraocular muscle is spared upon complete laminin alpha2 chain deficiency: Comparative expression of laminin and integrin isoforms.},
  url          = {http://dx.doi.org/10.1016/j.matbio.2006.05.001},
  volume       = {25},
  year         = {2006},
}