Chapter, 2024

Chapter 30 Elastin

Biochemistry of Collagens, Laminins and Elastin 9780443156175, Pages 279-289

Editors:

Publisher: Elsevier

DOI: 10.1016/b978-0-443-15617-5.00016-0

Contributors

Rønnow, Sarah Rank 0000-0002-9022-8775 [1] Kristensen, Jacob Hull Edfort 0000-0002-8467-0989 [1] Thorlacius-Ussing, Jeppe 0000-0002-3340-0786 [1] Karsdal, Morten Asser 0000-0002-4764-5100 [1] Heinz, Andrea 0000-0002-8609-4460 [2]

Affiliations

  1. [1] Nordic Bioscience (Denmark)
    2. [NORA names: Nordic Bioscience; Private Research; Denmark; Europe, EU; Nordic; OECD];
  2. [2] University of Copenhagen
    3. [NORA names: KU University of Copenhagen; University; Denmark; Europe, EU; Nordic; OECD]

Abstract

Elastin is a key extracellular matrix protein that is roughly a 1000 times more flexible than collagens. The main function of elastin is to provide elasticity and resilience to extensible tissues like the lungs, aorta, and skin. The precursor of elastin is tropoelastin, which is primarily produced in fibroblasts, smooth muscle cells, chondrocytes, and endothelial cells before being cross-linked to form mature elastin. Mutations in the elastin gene may lead to diseases such as Williams–Beuren syndrome, cutis laxa, and supravalvular aortic stenosis. Elastin degradation can release bioactive fragments with diverse signaling properties that can drive disease progression, as seen in cancer and emphysema. In addition, elastin degradation has been linked to chronic obstructive pulmonary disease, idiopathic pulmonary fibrosis, and cardiovascular disease. Several important and well-described biomarkers of elastin degradation are available.

Keywords

Williams-Beuren, Williams-Beuren syndrome, aorta, aortic stenosis, bioactive fragments, biomarkers, biomarkers of elastin degradation, cancer, cardiovascular disease, cells, chondrocytes, chronic obstructive pulmonary disease, collagen, cross-linking, cutis, cutis laxa, degradation, disease, disease progression, elasticity, elastin, elastin degradation, elastin gene, emphysema, endothelial cells, extracellular matrix proteins, fibroblasts, fibrosis, fragments, function, function of elastin, genes, idiopathic pulmonary fibrosis, laxa, lung, matrix proteins, mature elastin, muscle cells, mutations, obstructive pulmonary disease, precursor, precursor of elastin, progression, properties, protein, pulmonary disease, pulmonary fibrosis, resilience, signal, signaling properties, skin, smooth muscle cells, stenosis, supravalvular aortic stenosis, syndrome, tissue, tropoelastin

Data Provider: Digital Science

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