Clinical Outcomes of Elastin Fibre Defects

The permanganate-bisulfite-toluidine blue reaction is a highly selective and sensitive method for demonstrating elastic fibers under polarizing optics. Resilience and elastic recoil are properties conferred on all vertebrate elastic tissues by elastic fibers [1,2]. The amorphous component is made up of insoluble elastin, a highly cross-linked and hydrophobic protein assembled from soluble precursor proteins called “tropoelastins.” The microfibrillar component of elastic fibers is composed of several glycoproteins, of which the best known are the fibrillins (fibrillin-1 and fibrillin-2). Once synthesized in early development, elastic fibers undergo very little turnover in normal adult tissues, with the notable exception of the uterus. The elastic fibers deposited in one’s aorta as a young child, for example, are the same elastic fibers that one usually will die with. In a variety of elastic-tissue diseases, however, new elastic-fiber synthesis in adult tissue results in the aberrant accumulation of dysfunctional elastic fibers. Examples of such common disorders involving aberrant elastic-fiber assembly include emphysema, hypertension and aortic aneurysms. The various defects in the Expression, Structure, Assembly, Degradation of the constituent structural glycoprotein (or glycoproteins) of micro fibrils may be implicated in the causation of diseases associated with elastic fibres. It has been seen that a protein called fibulin-5, or DANCE (Developing Arteries and Neural Crest EGF-like) plays a substantial role in the generation and organization of elastic fibers in mice.