Biogenesis of extracellular microfibrils: Multimerization of the fibrillin-1 C terminus into bead-like structures enables self-assembly.

@article{Hubmacher2008BiogenesisOE,
  title={Biogenesis of extracellular microfibrils: Multimerization of the fibrillin-1 C terminus into bead-like structures enables self-assembly.},
  author={Dirk Hubmacher and Ehab I. El-Hallous and Valentin Nelea and Mari T Kaartinen and Eunice Rosalind Lee and Dieter P Reinhardt},
  journal={Proceedings of the National Academy of Sciences of the United States of America},
  year={2008},
  volume={105 18},
  pages={6548-53}
}
Microfibrils are essential elements in elastic and nonelastic tissues contributing to homeostasis and growth factor regulation. Fibrillins form the core of these multicomponent assemblies. Various human genetic disorders, the fibrillinopathies, arise from mutations in fibrillins and are frequently associated with aberrant microfibril assembly. These disorders include Marfan syndrome, Weill-Marchesani syndrome, Beals syndrome, and others. Although homotypic and heterotypic fibrillin self… CONTINUE READING

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