title: Role of the extracellular matrix and its receptors in smooth muscle cell function: implications in vascular development and disease.
abstract: OBJECTIVE
  Cardiovascular disease affects millions of people worldwide, while the sarcoglycan deficient cardiomyopathies are rare disorders. One important common feature, however, is the vascular smooth muscle. Here we focus on the roles of extracellular matrix components and their receptors in the functions of vascular smooth muscle cells.
  
  
  RESULTS
  Recent observations highlight the importance of integrins and the dystrophin-glycoprotein complex in development and cardiomyopathy. For example, integrin alpha4 and alpha7 subunits are important for distributing vascular smooth muscle cells during blood vessel development. Studies on delta-sarcoglycan deficient animals have revealed abnormal vascular smooth muscle proliferation and apoptosis. Furthermore, data suggest that perlecan, by affecting smooth muscle cell proliferation, participates in the atherosclerotic process. Overexpression of decorin leads to reduced progression of atherosclerosis and thrombospondin-1 has been implicated in regulation of smooth muscle cell contractility via inhibition of nitric oxide. Novel findings on versican suggest that the binding of versican to fibulin is of great importance for regulating smooth muscle cell function.
  
  
  CONCLUSIONS
  By regulating migration, proliferation and apoptosis as well as extracellular matrix synthesis and assembly, proteoglycans, integrins and the dystrophin-glycoprotein complex may be of great importance both during development and in vascular disease.
authors: Vessel Wall Biology Unit, Sweden bMuscle Biology Unit, University of Lund, Lund, Sweden. anna.hultgardh@med.lu.se
authors: Anna
authors: A
authors: Hultgårdh-Nilsson
authors: Madeleine
authors: M
authors: Durbeej
mesh_terms: D000818:Animals; D001808:Blood Vessels / Q000378:metabolism / Q000502:physiology*; D049030:Dystroglycans / Q000378:metabolism / Q000502:physiology; D005109:Extracellular Matrix / Q000378:metabolism / Q000502:physiology*; D019812:Heparan Sulfate Proteoglycans / Q000378:metabolism / Q000502:physiology; D006801:Humans; D007797:Laminin / Q000378:metabolism / Q000502:physiology; D008954:Models, Biological; D009131:Muscle, Smooth, Vascular / Q000166:cytology / Q000378:metabolism / Q000502:physiology*; D014652:Vascular Diseases / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*
chemical_list: D019812:Heparan Sulfate Proteoglycans; D007797:Laminin; C071927:perlecan; D049030:Dystroglycans