The Molecular Biology and Pathology of Elastic Tissues

This is a collection of recent research papers on the biology of membrane-associated elastin-binding proteins. It includes discussion of the structure and function of elastin, its role in ageing and pathology, its molecular biology and the regulation of expression of elastin and related genes.

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Elastin is a molecule of the extracellular matrix, which appeared during phylogenesis only with the vertebrates. Organs and tissues rich in elastin (e.g. in the circulatory and respiratory systems) age the most rapidly. Elastic fibres are complex structures and their biosynthesis presents problems for the elastin-producing cell. Physiological turnover of insoluble elastin is very slow, so little remodelling of elastic fibres occurs during adult life. However, increased elastin destruction occurs in certain pathological conditions, due either to the release of elastases from inflammatory cells or bacteria, or to a genetic deficiency in an elastase inhibitor. Membrane-associated elastin-binding proteins have been discovered recently. These have a high affinity for tropoelastin (the soluble elastin precursor) and for fragments of insoluble elastin. Also, they mediate a variety of cellular reactions, including chemotaxis, release of lytic enzymes or oxygen radicals, and cell adhesion. They may also play a role in the movement of inflammatory cells and metastic tumour cells through elastin-rich tissues.
With the cloning of the genes for tropoelastin and several microfibrillar proteins, as well as the discovery of elastin receptors, research on this topic is proceeding rapidly. This book includes contributions from a diverse group of scientists and clinicians working on elastin biology. Specific topics include the structure and function of elastin, its molecular biology and regulation, and its role in ageing and pathology.