Elastic fibers during aging and disease

Abstract

Elastic fibers are essential constituents of the extracellular matrix of higher vertebrates and endow several tissues and organs including lungs, skin and blood vessels with elasticity and resilience. During the human lifespan, elastic fibers are exposed to a variety of enzymatic, chemical and biophysical influences, and accumulate damage due to their low turnover. Aging of elastin and elastic fibers involves enzymatic degradation, oxidative damage, glycation, calcification, aspartic acid racemization, binding of lipids and lipid peroxidation products, carbamylation and mechanical fatigue. These processes can trigger an impairment or loss of elastic fiber function and are associated with severe pathologies. There are different inherited or acquired pathological conditions, which influence the structure and function of elastic fibers and microfibrils predominantly in the cardiorespiratory system and skin. Inherited elastic-fiber pathologies have a direct or indirect impact on elastic-fiber formation due to mutations in the fibrillin genes (fibrillinopathies), in the elastin gene (elastinopathies) or in genes encoding proteins that are associated with microfibrils or elastic fibers. Acquired elastic-fiber pathologies appear age-related or as a result of multiple factors impairing tissue homeostasis. This review gives an overview on the fate of elastic fibers over the human lifespan in health and disease.

Keywords: Calcification; Elastokines; Fibrillin; Fibrillinopathies; Glycation; Microfibrils.