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Laminin alpha1 chain mediated reduction of laminin alpha2 chain deficient muscular dystrophy involves integrin alpha7beta1 and dystroglycan.

Gawlik, Kinga LU ; Mayer, Ulrike; Blomberg, Kristina LU ; Sonnenberg, Arnoud; Ekblom, Peter LU and Durbeej-Hjalt, Madeleine LU (2006) In FEBS Letters 580(7). p.1759-1765
Abstract
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.
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Integrin, Dystroglycan, Laminin, Muscular dystrophy
in
FEBS Letters
volume
580
issue
7
pages
1759 - 1765
publisher
Wiley-Blackwell
external identifiers
  • wos:000236338300011
  • pmid:16504180
  • scopus:33644892480
ISSN
1873-3468
DOI
10.1016/j.febslet.2006.02.027
language
English
LU publication?
yes
id
dcb29022-18a7-4c96-895c-b431d6342146 (old id 154948)
alternative location
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16504180&dopt=Abstract
date added to LUP
2007-07-12 12:52:36
date last changed
2019-09-26 02:40:17
@article{dcb29022-18a7-4c96-895c-b431d6342146,
  abstract     = {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.},
  author       = {Gawlik, Kinga and Mayer, Ulrike and Blomberg, Kristina and Sonnenberg, Arnoud and Ekblom, Peter and Durbeej-Hjalt, Madeleine},
  issn         = {1873-3468},
  keyword      = {Integrin,Dystroglycan,Laminin,Muscular dystrophy},
  language     = {eng},
  number       = {7},
  pages        = {1759--1765},
  publisher    = {Wiley-Blackwell},
  series       = {FEBS Letters},
  title        = {Laminin alpha1 chain mediated reduction of laminin alpha2 chain deficient muscular dystrophy involves integrin alpha7beta1 and dystroglycan.},
  url          = {http://dx.doi.org/10.1016/j.febslet.2006.02.027},
  volume       = {580},
  year         = {2006},
}