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Elastin in lung development and disease pathogenesis.

Robert P Mecham1

  • 1Department of Cell Biology & Physiology, Washington University School of Medicine, St. Louis, MO, USA.

Matrix Biology : Journal of the International Society for Matrix Biology
|January 15, 2018
PubMed
Summary
This summary is machine-generated.

Elastin, a key protein for elastic recoil, is vital for lung function and overall vertebrate evolution. Its proper assembly in the extracellular matrix is essential for maintaining lung health and preventing disease.

Keywords:
Elastic fiberElastinEmphysemaFibrillinLung developmentMicrofibril

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Area of Science:

  • Biochemistry
  • Cell Biology
  • Evolutionary Biology

Background:

  • Elastin provides elastic recoil in tissues, crucial for vertebrate evolution and the circulatory system.
  • In the lung, elastin is produced by various cells and forms elastic fibers in the extracellular matrix.
  • Elastic fibers are essential for lung structure and function, particularly in pulmonary vessels and the broader lung network.

Purpose of the Study:

  • To highlight the critical role of elastin in lung physiology and disease.
  • To describe the expression and structural contribution of elastin in the pulmonary system.
  • To emphasize the necessity of proper elastin assembly for normal lung function.

Main Methods:

  • Literature review on elastin expression and function.
  • Analysis of elastin's role in tissue mechanics and evolution.
  • Examination of elastin's contribution to lung structure and disease pathogenesis.

Main Results:

  • Elastin is expressed by multiple lung cell types, including smooth muscle and interstitial fibroblasts.
  • Elastic fibers form diverse structures, such as lamellae in vessels and a network in lung parenchyma.
  • Elastin assembly is critical for transmitting force equally throughout the lung.

Conclusions:

  • Proper secretion and assembly of elastin are fundamental for normal lung function.
  • Disruption of elastin fiber assembly or degradation leads to lung dysfunction and disease.
  • Elastin's evolutionary significance is underscored by its role in the development of the closed circulatory system.