RGD-containing fibrillin-1 fragments upregulate matrix metalloproteinase expression in cell culture: A potential factor in the pathogenesis of the Marfan syndrome

@article{Booms2004RGDcontainingFF,
  title={RGD-containing fibrillin-1 fragments upregulate matrix metalloproteinase expression in cell culture: A potential factor in the pathogenesis of the Marfan syndrome},
  author={Patrick Booms and Reinhard Pregla and Andreas Ney and Frank Barthel and Dieter P Reinhardt and Angelika Pletschacher and Stefan Mundlos and Peter N. Robinson},
  journal={Human Genetics},
  year={2004},
  volume={116},
  pages={51-61}
}
The Marfan syndrome (MFS), a relatively common autosomal dominant disorder of connective tissue, is caused by mutations in the gene for fibrillin-1 (FBN1). Fibrillin-1 is the main component of the 10- to 12-nm microfibrils that together with elastin form elastic fibers found in tissues such as the aortic media. Recently, FBN1 mutations have been shown to increase the susceptibility of fibrillin-1 to proteolysis in vitro, and other findings suggest that up-regulation of matrix metalloproteinases… CONTINUE READING
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