title: Increased neointimal thickening in dystrophin-deficient mdx mice.
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.
  
  
  RESULTS
  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
  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.
authors.affiliation: Department of Experimental Medical Science, Lund University, Lund, Sweden.
authors.forename: Uwe
authors.initials: U
authors.lastname: Rauch
authors.forename: Annelie
authors.initials: A
authors.lastname: Shami
authors.forename: Feng
authors.initials: F
authors.lastname: Zhang
authors.forename: Virginie
authors.initials: V
authors.lastname: Carmignac
authors.forename: Madeleine
authors.initials: M
authors.lastname: Durbeej
authors.forename: Anna
authors.initials: A
authors.lastname: Hultgårdh-Nilsson
mesh_terms: D000818:Animals; D049109:Cell Proliferation; D002478:Cells, Cultured; D016189:Dystrophin / Q000172:deficiency / Q000235:genetics* / Q000378:metabolism / Q000502:physiology; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D009131:Muscle, Smooth, Vascular / Q000378:metabolism / Q000473:pathology; D009137:Muscular Dystrophy, Animal / Q000150:complications / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D020388:Muscular Dystrophy, Duchenne / Q000150:complications / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D032389:Myocytes, Smooth Muscle / Q000378:metabolism / Q000473:pathology; D058426:Neointima / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D009929:Organ Size; D015854:Up-Regulation; D057772:Vascular System Injuries / Q000235:genetics / Q000378:metabolism / Q000473:pathology
chemical_list: D016189:Dystrophin