| Small leucine-rich proteoglycans (SLRPs) such as Decorin, Biglycan, Fibromod-ulin, Keratocan, Lumican and Osteoglycin mediate extracellular matrix organization and are binding partners of TGF Beta. The Decorin core protein binds to growth factors, intercellular matrix molecules such as Fibronectin and Throm-bospondin, and to the Decorin endocytosis receptor. Fibromodulin is a collagen-binding keratan sulphate proteoglycan that influences adhesion processes of connective tissue and plays a role in fibrillogenesis by regulating collagen fibril spacing and thickness. Keratocan (KTN) develops corneal transparency and maintains the stromal matrix structure. Keratocan is a secreted protein in the extracellular matrix that binds to keratan sulfate chains. Keratocan is mainly detected in the cornea, but can also be expressed in trachea, intestine, ovary, lung and skeletal muscle. Defects in the gene encoding for Keratocan can cause cornea plana 2 (CNA2), an autosomal recessive disorder where the forward convex curvature of the cornea is flattened. |
Powered by Bioz