Fibulins: Physiological and Disease Perspectives

EMBO Rep. 2003 Dec;4(12):1127-31. doi: 10.1038/sj.embor.7400033.

Abstract

The fibulins are a family of proteins that are associated with basement membranes and elastic extracellular matrix fibres. This review summarizes findings from studies of animal models of fibulin deficiency, human fibulin gene mutations, human tumours and injury models that have advanced our understanding of the normal and pathological roles of members of this formerly obscure family.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Basement Membrane / metabolism
  • Calcium-Binding Proteins / chemistry
  • Calcium-Binding Proteins / genetics
  • Calcium-Binding Proteins / physiology*
  • Cardiovascular System / metabolism
  • Elastic Tissue / metabolism
  • Extracellular Matrix Proteins / genetics
  • Extracellular Matrix Proteins / metabolism
  • Extracellular Matrix Proteins / physiology
  • Gene Expression Regulation
  • Humans
  • Mice
  • Mice, Knockout
  • Models, Animal
  • Molecular Sequence Data
  • Neoplasms / genetics
  • Neoplasms / metabolism
  • Recombinant Proteins
  • Wounds and Injuries / genetics
  • Wounds and Injuries / metabolism

Substances

  • Calcium-Binding Proteins
  • Extracellular Matrix Proteins
  • FBLN5 protein, human
  • Fbln5 protein, mouse
  • Recombinant Proteins
  • fibulin
  • fibulin 2
  • fibulin-4