New insights into elastic fiber assembly

Birth Defects Res C Embryo Today. 2007 Dec;81(4):229-40. doi: 10.1002/bdrc.20111.

Abstract

Elastic fibers provide recoil to tissues that undergo repeated stretch, such as the large arteries and lung. These large extracellular matrix (ECM) structures contain numerous components, and our understanding of elastic fiber assembly is changing as we learn more about the various molecules associated with the assembly process. The main components of elastic fibers are elastin and microfibrils. Elastin makes up the bulk of the mature fiber and is encoded by a single gene. Microfibrils consist mainly of fibrillin, but also contain or associate with proteins such as microfibril associated glycoproteins (MAGPs), fibulins, and EMILIN-1. Microfibrils were thought to facilitate alignment of elastin monomers prior to cross-linking by lysyl oxidase (LOX). We now know that their role, as well as the overall assembly process, is more complex. Elastic fiber formation involves elaborate spatial and temporal regulation of all of the involved proteins and is difficult to recapitulate in adult tissues. This report summarizes the known interactions between elastin and the microfibrillar proteins and their role in elastic fiber assembly based on in vitro studies and evidence from knockout mice. We also propose a model of elastic fiber assembly based on the current data that incorporates interactions between elastin, LOXs, fibulins and the microfibril, as well as the pivotal role played by cells in structuring the final functional fiber.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Calcium-Binding Proteins / genetics
  • Calcium-Binding Proteins / metabolism
  • Contractile Proteins / genetics
  • Contractile Proteins / metabolism
  • Elastic Tissue / growth & development
  • Elastic Tissue / metabolism*
  • Elastic Tissue / ultrastructure
  • Elastin / deficiency
  • Elastin / genetics
  • Elastin / metabolism
  • Extracellular Matrix Proteins / genetics
  • Extracellular Matrix Proteins / metabolism
  • Fibrillins
  • Gene Expression Regulation, Developmental
  • Humans
  • Membrane Glycoproteins / genetics
  • Membrane Glycoproteins / metabolism
  • Mice
  • Mice, Knockout
  • Microfilament Proteins / genetics
  • Microfilament Proteins / metabolism
  • Models, Biological
  • Mutation
  • Protein-Lysine 6-Oxidase / metabolism
  • RNA Splicing Factors
  • Rats
  • Tropoelastin / genetics
  • Tropoelastin / metabolism

Substances

  • Calcium-Binding Proteins
  • Contractile Proteins
  • Extracellular Matrix Proteins
  • Fibrillins
  • Membrane Glycoproteins
  • Microfilament Proteins
  • RNA Splicing Factors
  • Tropoelastin
  • elastin microfibril interface located protein
  • fibulin
  • microfibrillar protein
  • Elastin
  • Protein-Lysine 6-Oxidase