Fibrillins can co-assemble in fibrils, but fibrillin fibril composition displays cell-specific differences

Noe L. Charbonneau, Bette J. Dzamba, Robert N. Ono, Douglas R. Keene, Glen M. Corson, Dieter P. Reinhardt, Lynn Y. Sakai

Research output: Contribution to journalArticle

96 Scopus citations

Abstract

Fibrillins are micro fibril-forming extracellular matrix macromolecules that modulate skeletal development. In humans, mutations in fibrillins result in long bone over-growth as well as other distinct phenotypes. Whether fibrillins form independent microfibrillar networks or can copolymerize, forming a single microfibril, is not known. However, this knowledge is required to determine whether phenotypes arise because of loss of singular or composite functions of fibrillins. Immunolocalization experiments using tissues and de novo matrices elaborated by cultured cells demonstrated that both fibrillins can be present in the same individual microfibril in certain tissues and that both fibrillins can copolymerize in fibroblast cultures. These studies suggest that the molecular information directing fibrillin fibril formation may be similar in both fibrillins. Furthermore, these studies provide a molecular basis for compensation of one fibrillin by the other during fetal life. In postnatal tissues, fibrillin-2 antibodies demonstrated exuberant staining in only one location: peripheral nerves. This surprising finding implicates distinct functions for fibrillin-2 in peripheral nerves, because a unique feature in humans and in mice mutant for fibrillin-2 is joint contractures that resolve over time.

Original languageEnglish (US)
Pages (from-to)2740-2749
Number of pages10
JournalJournal of Biological Chemistry
Volume278
Issue number4
DOIs
StatePublished - Jan 24 2003

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology
  • Cell Biology

Fingerprint Dive into the research topics of 'Fibrillins can co-assemble in fibrils, but fibrillin fibril composition displays cell-specific differences'. Together they form a unique fingerprint.

  • Cite this