Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices

• DOI: 10.1083/jcb.124.5.855
• Publication Date: 2004-05-15T00:22:22Z
• Authors: H Zhang; SD Apfelroth; W Hu; EC Davis; C Sanguineti; J Bonadio; RP Mecham; F Ramirez
• Keywords: Fibrillin; Microfibril; Immunoelectron microscopy

During the previous cloning of fibrillin gene (FBN1), we isolated a partial cDNA coding for fibrillin-like peptide and mapped corresponding (FBN2) to human chromosome 5. (Lee, B., M. Godfrey, E. Vitale, H. Hori, G. Mattei, Sarfarazi, P. Tsipouras, F. Ramirez, D. W. Hollister. 1991. Nature [Lond.]. 352:330-334). The study left, however, unresolved whether or not FBN2 product is an extracellular component structurally related fibrillin. Work presented in this report clarifies important point. Determination entire primary structure demonstrated that polypeptide highly homologous Immunoelectron microscopy localized both proteins elastin-associated microfibrils. Finally, immunohistochemistry revealed fibrillins co-distribute elastic non-elastic connective tissues developing embryo, with preferential accumulation fiber-rich matrices. These results support original hypothesis may have distinct but functions formation maintenance Accordingly, propose classify FBN1 products as new family name its members fibrillin-1 fibrillin-2, respectively.