title: Structure and expression of fibrillin-2, a novel microfibrillar component preferentially located in elastic matrices.
abstract: During the previous cloning of the fibrillin gene (FBN1), we isolated a partial cDNA coding for a fibrillin-like peptide and mapped the corresponding gene (FBN2) to human chromosome 5. (Lee, B., M. Godfrey, E. Vitale, H. Hori, M. G. Mattei, M. Sarfarazi, P. Tsipouras, F. Ramirez, and D. W. Hollister. 1991. Nature [Lond.]. 352:330-334). The study left, however, unresolved whether or not the FBN2 gene product is an extracellular component structurally related to fibrillin. Work presented in this report clarifies this important point. Determination of the entire primary structure of the FBN2 gene product demonstrated that this polypeptide is highly homologous to fibrillin. Immunoelectron microscopy localized both fibrillin proteins to elastin-associated extracellular microfibrils. Finally, immunohistochemistry revealed that the fibrillins co-distribute in elastic and non-elastic connective tissues of the developing embryo, with preferential accumulation of the FBN2 gene product in elastic fiber-rich matrices. These results support the original hypothesis that the fibrillins may have distinct but related functions in the formation and maintenance of extracellular microfibrils. Accordingly, we propose to classify the FBN1 and FBN2 gene products as a new family of extracellular proteins and to name its members fibrillin-1 and fibrillin-2, respectively.
authors.affiliation: Brookdale Center for Molecular Biology, Mt. Sinai School of Medicine, New York 10029-6574.
authors.forename: H
authors.initials: H
authors.lastname: Zhang
authors.forename: S D
authors.initials: SD
authors.lastname: Apfelroth
authors.forename: W
authors.initials: W
authors.lastname: Hu
authors.forename: E C
authors.initials: EC
authors.lastname: Davis
authors.forename: C
authors.initials: C
authors.lastname: Sanguineti
authors.forename: J
authors.initials: J
authors.lastname: Bonadio
authors.forename: R P
authors.initials: RP
authors.lastname: Mecham
authors.forename: F
authors.initials: F
authors.lastname: Ramirez
mesh_terms: D000595:Amino Acid Sequence; D015152:Blotting, Northern; D002460:Cell Line; D002874:Chromosome Mapping; D002895:Chromosomes, Human, Pair 5; D003001:Cloning, Molecular; D003545:Cysteine / Q000378:metabolism; D018076:DNA, Complementary / Q000378:metabolism; D005333:Fetus / Q000166:cytology / Q000378:metabolism*; D000071838:Fibrillin-1; D000071840:Fibrillin-2; D000071837:Fibrillins; D015870:Gene Expression; D015723:Gene Library; D006801:Humans; D007150:Immunohistochemistry; D008840:Microfilament Proteins / Q000096:biosynthesis* / Q000737:chemistry* / Q000235:genetics; D016253:Microscopy, Immunoelectron; D008969:Molecular Sequence Data; D009928:Organ Specificity; D017386:Sequence Homology, Amino Acid; D054392:Adipokines
chemical_list: D003545:Cysteine; D018076:DNA, Complementary; D000071838:Fibrillin-1; D000071840:Fibrillin-2; D000071837:Fibrillins; D008840:Microfilament Proteins; C000605722:FBN1 protein, human; D054392:Adipokines; C000605723:FBN2 protein, human