作者: Jouni Uitto
DOI: 10.1111/1523-1747.EP12530093
关键词: Elastin 、 Dense connective tissue 、 Biochemistry 、 Elastic fiber 、 Fibrillogenesis 、 Connective tissue 、 Cutis laxa 、 Chemistry 、 Elastosis perforans serpiginosa 、 Fibrillin
摘要: The elastic fibers present in various connective tissues of the body are responsible for physiologic elasticity organs. These consist 2 distinct components, elastin and fiber microfibrils. Controlled synthesis balanced interaction these components essential normal fibrillogenesis. intracellular biosynthesis by tissue cells, such as smooth muscle involves assembly polypeptide chains on membrane-bound ribosomes, hydroxylation some prolyl residues to hydroxyproline, secretion polypeptides packaged Golgi vacuoles. In extracellular space molecules assemble into structures which stabilized complex covalent cross-links, desmosines. Recently, aberrations structure or metabolism have been detected a variety heritable acquired diseases affecting skin other tissues. conditions include pseudoxanthoma elasticum, cutis laxa, elastosis perforans serpiginosa, well arteriosclerosis degenerative changes vascular