Quantitative differences in biosynthesis and extracellular deposition of fibrillin in cultured fibroblasts distinguish five groups of Marfan syndrome patients and suggest distinct pathogenetic mechanisms.

Ítems similars

• Marfan syndrome: defective synthesis, secretion, and extracellular matrix formation of fibrillin by cultured dermal fibroblasts.
  per: Milewicz, D M, et al.
  Publicat: (1992)
• Expression of a mutant human fibrillin allele upon a normal human or murine genetic background recapitulates a Marfan cellular phenotype.
  per: Eldadah, Z A, et al.
  Publicat: (1995)
• ADAMTS10 Protein Interacts with Fibrillin-1 and Promotes Its Deposition in Extracellular Matrix of Cultured Fibroblasts
  per: Kutz, Wendy E., et al.
  Publicat: (2011)
• Deficiencies of fibrillin and decorin in fibroblast cultures of a patient with neonatal Marfan syndrome.
  per: Superti-Furga, A, et al.
  Publicat: (1992)
• Abnormal fibrillin assembly by dermal fibroblasts from two patients with Marfan syndrome
  Publicat: (1994)