title: [Sulfated macromolecules in connective tissue and their metabolism].
abstract: Sulfated macromolecules are one of the major constituents of the extracellular matrix of connective tissue. The degree of sulfation is responsible for the physico-chemical properties of the extracellular matrix. Especially the fixed charged groups contribute to the reversible compressibility of the cartilage. The sulfation may play also a role in the regulation of metabolic pathways. The derivatives of sulfate are formed by the help of specific sulfotransferases, transferring the "active" sulfate group from PAPS to the macromolecules. Too low an activity of certain sulfotransferases is responsible for some types of spondyloepiphyseal dysplasia, and too low a concentration of PAPS leads to dwarfism. The sulfate groups of the macromolecules are degraded by specific sulfatases. A genetically caused deficiency in one of these enzymes (mucopolysaccharidoses) is frequently associated with skeletal deformities. The amount and composition of proteoglycans is altered in the ontogenesis. Similar alterations are associated with ageing and arthrotic processes, being difficult to differentiate. The characterization of the alterations in degenerative joint diseases, especially, is important for ethiopathogenetic studies. The most widely used methods for the determination of sulfated macromolecules and particularly analytical parameters of the sulfate metabolism are described.
mesh_terms: D001192:Arylsulfatases / Q000378:metabolism; D003238:Connective Tissue / Q000378:metabolism*; D006025:Glycosaminoglycans / Q000378:metabolism; D006801:Humans; D007700:Kinetics; D011509:Proteoglycans / Q000378:metabolism; D013464:Sulfuric Acids / Q000378:metabolism*; D013466:Sulfurtransferases / Q000172:deficiency / Q000378:metabolism*
chemical_list: D006025:Glycosaminoglycans; D011509:Proteoglycans; D013464:Sulfuric Acids; D013466:Sulfurtransferases; D001192:Arylsulfatases
vernacular_title: Sulfatierte Makromoleküle des Bindegewebes und ihr Stoffwechsel.