Extraocular muscle is spared upon complete laminin alpha2 chain deficiency: Comparative expression of laminin and integrin isoforms.
(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:
https://lup.lub.lu.se/record/158261
- author
- Nyström, Alexander LU ; Holmblad, Johanna ; Pedrosa-Domellof, Fatima ; Sasaki, Takako and Durbeej-Hjalt, Madeleine LU
- organization
- publishing date
- 2006
- 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}}, }