Advanced

Increased Neointimal Thickening in Dystrophin-Deficient mdx Mice.

Rauch, Uwe LU ; Shami, Annelie LU ; Zhang, Feng LU ; Carmignac, Virginie LU ; Durbeej-Hjalt, Madeleine LU and Hultgårdh, Anna LU (2012) In PLoS ONE 7(1).
Abstract
BACKGROUND:

The dystrophin gene, which is mutated in Duchenne muscular dystrophy (DMD), encodes a large cytoskeletal protein present in muscle fibers. While dystrophin in skeletal muscle has been extensively studied, the function of dystrophin in vascular smooth muscle is less clear. Here, we have analyzed the role of dystrophin in injury-induced arterial neointima formation.

METHODOLOGY/PRINCIPAL FINDINGS:

We detected a down-regulation of dystrophin, dystroglycan and β-sarcoglycan mRNA expression when vascular smooth muscle cells de-differentiate in vitro. To further mimic development of intimal lesions, we performed a collar-induced injury of the carotid artery in the mdx mouse, a model for DMD. As compared... (More)
BACKGROUND:

The dystrophin gene, which is mutated in Duchenne muscular dystrophy (DMD), encodes a large cytoskeletal protein present in muscle fibers. While dystrophin in skeletal muscle has been extensively studied, the function of dystrophin in vascular smooth muscle is less clear. Here, we have analyzed the role of dystrophin in injury-induced arterial neointima formation.

METHODOLOGY/PRINCIPAL FINDINGS:

We detected a down-regulation of dystrophin, dystroglycan and β-sarcoglycan mRNA expression when vascular smooth muscle cells de-differentiate in vitro. To further mimic development of intimal lesions, we performed a collar-induced injury of the carotid artery in the mdx mouse, a model for DMD. As compared with control mice, mdx mice develop larger lesions with increased numbers of proliferating cells. In vitro experiments demonstrate increased migration of vascular smooth muscle cells from mdx mice whereas the rate of proliferation was similar in cells isolated from wild-type and mdx mice.

CONCLUSIONS/SIGNIFICANCE:

These results show that dystrophin deficiency stimulates neointima formation and suggest that expression of dystrophin in vascular smooth muscle cells may protect the artery wall against injury-induced intimal thickening. (Less)
Please use this url to cite or link to this publication:
author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
PLoS ONE
volume
7
issue
1
publisher
Public Library of Science
external identifiers
  • wos:000301070200057
  • pmid:22238670
  • scopus:84855388573
ISSN
1932-6203
DOI
10.1371/journal.pone.0029904
language
English
LU publication?
yes
id
9f1a61d3-d5ba-48fd-8ded-6619ed1d1dd8 (old id 2336489)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/22238670?dopt=Abstract
date added to LUP
2012-02-01 20:38:43
date last changed
2017-09-17 06:22:50
@article{9f1a61d3-d5ba-48fd-8ded-6619ed1d1dd8,
  abstract     = {BACKGROUND:<br/><br>
The dystrophin gene, which is mutated in Duchenne muscular dystrophy (DMD), encodes a large cytoskeletal protein present in muscle fibers. While dystrophin in skeletal muscle has been extensively studied, the function of dystrophin in vascular smooth muscle is less clear. Here, we have analyzed the role of dystrophin in injury-induced arterial neointima formation.<br/><br>
METHODOLOGY/PRINCIPAL FINDINGS:<br/><br>
We detected a down-regulation of dystrophin, dystroglycan and β-sarcoglycan mRNA expression when vascular smooth muscle cells de-differentiate in vitro. To further mimic development of intimal lesions, we performed a collar-induced injury of the carotid artery in the mdx mouse, a model for DMD. As compared with control mice, mdx mice develop larger lesions with increased numbers of proliferating cells. In vitro experiments demonstrate increased migration of vascular smooth muscle cells from mdx mice whereas the rate of proliferation was similar in cells isolated from wild-type and mdx mice.<br/><br>
CONCLUSIONS/SIGNIFICANCE:<br/><br>
These results show that dystrophin deficiency stimulates neointima formation and suggest that expression of dystrophin in vascular smooth muscle cells may protect the artery wall against injury-induced intimal thickening.},
  articleno    = {e29904},
  author       = {Rauch, Uwe and Shami, Annelie and Zhang, Feng and Carmignac, Virginie and Durbeej-Hjalt, Madeleine and Hultgårdh, Anna},
  issn         = {1932-6203},
  language     = {eng},
  number       = {1},
  publisher    = {Public Library of Science},
  series       = {PLoS ONE},
  title        = {Increased Neointimal Thickening in Dystrophin-Deficient mdx Mice.},
  url          = {http://dx.doi.org/10.1371/journal.pone.0029904},
  volume       = {7},
  year         = {2012},
}