Fibrillin-rich microfibrils: Structural determinants of morphogenetic and homeostatic events.

  title={Fibrillin-rich microfibrils: Structural determinants of morphogenetic and homeostatic events.},
  author={Francesco Ramirez and Harry C Dietz},
  journal={Journal of cellular physiology},
  volume={213 2},
Fibrillin-rich microfibrils are specialized extracellular matrix assemblies that endow connective tissues with mechanical stability and elastic properties, and that participate in the regulation of organ formation, growth and homeostasis. Their physiological importance is underscored by the complex spectrum of clinical manifestations associated with mutations of fibrillin-1 and fibrillin-2 in Marfan syndrome (MFS) and congenital contractural arachnodactyly, respectively. Early evidence… CONTINUE READING


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