Abstract

The Gordon Research Conference (GRC) and the Gordon Research Seminar (GRS) on Elastin, Elastic Fibers and Microfibrils will focus on elastic fiber molecules and the roles that elastic fibers and related fiber systems exert in human disease, vertebrate development, tissue homeostasis and aging. Elastin, elastic fibers and microfibrils are critical components of the skin, bone, vasculature, lung, tendons, eye and other tissues. Elastin fibers are unique because they provide elasticity to many dynamic tissues, i.e., deforms under physiological forces and subsequently releases stored energy to drive passive recoil. This conference will focus on elastic tissue biology and topics include: molecular properties of elastin, fibrillin and other proteins essential for the assembly and function of elastic fibers; elastic tissue matrix regulation of cell signaling; mechanosensing of the elastin-smooth muscle cell complex; aging and stiffening of elastin-containing tissues; technologies for elastic fiber system analysis; genetics and pathobiology of elastic fiber associated molecules in inherited and acquired disorders; tissue regeneration, bioengineering of elastic tissues; and application to translational medicine. The conference will provide new insights into the mechanisms underlying the normal function of elastic fiber-rich tissues and explore new therapeutic avenues for genetic and acquired disorders originating from understanding the detailed functions of the elastic fiber system. The elastic fiber-rich musculoskeletal, cardiovascular, and pulmonary tissues are a major focus. Disorders discussed during the conference that are directly relevant to these systems include but are not limited to aneurysms and dissections, aortic stiffening, aortic calcification, heritable thoracic aortic disease, Marfan syndrome, Marfanoid-progeroid-lipodystrophy, Loeys-Dietz syndrome, supravalvular aortic stenosis, Williams-Beuren syndrome, arterial tortuosity syndrome, chronic obstructive pulmonary disease (COPD), acromicric dysplasia and geleophysic dysplasia, Weill-Marchesani syndrome, Urban-Rifkin-Davis syndrome, cutis laxa, pseudoxanthoma elasticum, glaucoma, and loss of elasticity in skin during normal and UV-induced photo-aging. These acquired and inherited diseases affect millions of Americans and hundreds of millions worldwide.