Müller G, Heilmann H H
Biomed Biochim Acta. 1984;43(7):867-86.
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.
硫酸化大分子是结缔组织细胞外基质的主要成分之一。硫酸化程度决定了细胞外基质的物理化学性质。尤其是固定电荷基团对软骨的可逆压缩性有影响。硫酸化在代谢途径的调节中也可能起作用。硫酸盐衍生物是在特定硫酸转移酶的作用下形成的,该酶将“活性”硫酸基团从PAPS转移到大分子上。某些硫酸转移酶活性过低会导致某些类型的脊椎骨骺发育不良,而PAPS浓度过低会导致侏儒症。大分子的硫酸基团由特定的硫酸酯酶降解。这些酶之一(黏多糖贮积症)的遗传缺陷常与骨骼畸形有关。蛋白聚糖的数量和组成在个体发育过程中会发生改变。类似的改变与衰老和关节病变有关,难以区分。特别是,退行性关节疾病中改变的特征对于病因发病机制研究很重要。本文描述了测定硫酸化大分子以及硫酸代谢分析参数的最常用方法。