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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)
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author
; ; ; and
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
additional info
The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Cell and Matrix Biology (LUR000002), Muscle biology (013212015)
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
2016-04-01 16:07:18
date last changed
2022-04-22 19:47:53
@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}},
  keywords     = {{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}},
  doi          = {{10.1016/j.matbio.2006.05.001}},
  volume       = {{25}},
  year         = {{2006}},
}