Heritable Connective Tissue Disorders
The extracellular matrix of connective tissue of the skin consists of a large number of distinct matrix macromolecules which play a critical role in providing physiologic properties to normal skin. Within the dermis, collagen and elastic fibers are the two major fibrillar components of connective tissue, and they exist in a fibrous interwoven mesh- work structure associated with other extracellular macromolecules of the dermis, such as proteoglycan/glycosaminoglycan complexes, and a number of non-collagenous glycoproteins, including fibronectin and fibrillins (Tables 1 and 2). Encased in the fibrillar mesh- work are cells that interact with the extracellular matrix components through cell-matrix receptors, such as integrins.
KeywordsElastic Fiber Marfan Syndrome Bullous Pemphigoid Epidermolysis Bullosa Pulmonary Emphysema
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