Increased Neointimal Thickening in Dystrophin-Deficient mdx Mice.

Rauch, UweLund University,Lunds universitet,Kärlväggsbiologi,Forskargrupper vid Lunds universitet,Vessel Wall Biology,Lund University Research Groups (author)

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.

Shami, AnnelieLund University,Lunds universitet,Kärlväggsbiologi,Forskargrupper vid Lunds universitet,Vessel Wall Biology,Lund University Research Groups(Swepub:lu)med-ais (author)
Zhang, FengLund University,Lunds universitet,Kärlväggsbiologi,Forskargrupper vid Lunds universitet,Muskelbiologi,Vessel Wall Biology,Lund University Research Groups,Muscle Biology(Swepub:lu)med-fgz (author)
Carmignac, VirginieLund University,Lunds universitet,Muskelbiologi,Forskargrupper vid Lunds universitet,Muscle Biology,Lund University Research Groups(Swepub:lu)med-vic (author)
Durbeej-Hjalt, MadeleineLund University,Lunds universitet,Muskelbiologi,Forskargrupper vid Lunds universitet,Muscle Biology,Lund University Research Groups(Swepub:lu)medk-mdu (author)
Hultgårdh, AnnaLund University,Lunds universitet,Kärlväggsbiologi,Forskargrupper vid Lunds universitet,Vessel Wall Biology,Lund University Research Groups(Swepub:lu)medk-ahu (author)
KärlväggsbiologiForskargrupper vid Lunds universitet (creator_code:org_t)

By the author/editor: Rauch, Uwe; Shami, Annelie; Zhang, Feng; Carmignac, Virgi ...; Durbeej-Hjalt, M ...; Hultgårdh, Anna

About the subject

MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Basic Medicine; and Cell and Molecul ...

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