elastic fiber assembly

Definition

Target type: biologicalprocess

Assembly of the extracellular matrix fibers that enables the matrix to recoil after transient stretching. [GOC:jid, PMID:10841810, PMID:12615674]

Elastic fiber assembly is a complex process involving the coordinated synthesis, secretion, and assembly of two major protein components: elastin and fibrillin-1. Elastin, a highly hydrophobic protein, provides the rubber-like elasticity to the fibers. Fibrillin-1, a large glycoprotein, forms a scaffold that guides elastin deposition and provides structural integrity to the fiber. The process begins with the synthesis of proelastin, the precursor to elastin, in fibroblasts and smooth muscle cells. Proelastin is then secreted into the extracellular matrix, where it undergoes a series of modifications including cleavage of its N-terminal propeptide and cross-linking of lysine residues by lysyl oxidase. This cross-linking forms desmosine and isodesmosine, unique amino acids that contribute to the elasticity and strength of elastin. Meanwhile, fibrillin-1 molecules assemble into microfibrils, which serve as a template for elastin deposition. These microfibrils interact with other extracellular matrix components, such as microfibril-associated glycoprotein (MAGP) and fibulin-5, to form a complex network that provides structural support and anchors the elastic fibers to surrounding tissues. As proelastin is deposited onto the microfibril scaffold, it undergoes further processing and cross-linking to form mature elastin fibers. This process requires the presence of specific enzymes, including lysyl oxidase and elastin-binding protein, which facilitate the assembly and stabilization of the fibers. The final mature elastic fibers are highly organized structures that exhibit a characteristic wavy appearance in electron microscopy. This morphology allows them to stretch and recoil, providing the tissues with their characteristic elastic properties. Defects in the genes encoding elastin, fibrillin-1, or other proteins involved in elastic fiber assembly can lead to a variety of connective tissue disorders, including Marfan syndrome, Cutis laxa, and Williams syndrome. These disorders are often characterized by weakened or abnormally structured elastic fibers, leading to impaired tissue function and structural integrity.'
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Proteins (1)

Compounds (10)