von Figura K
Hautarzt. 1976 May;27(5):206-13.
The basic defects of a number of genetic disorders affecting the connective tissue have been elucidiated during the last few years. A similar progress in understanding the pathobiochemistry of other dermatological diseases is expected. The present review summarizes our knowledge about structure, synthesis, degradation, macromolecular organization and possible function of glycosaminoglycans, which are an important constitutent of the ground substance in the skin. The difficulties of relating the clinical symptoms of diseases with the basic defects are examplified for the mucopolysaccharidoses.
在过去几年中,已经阐明了一些影响结缔组织的遗传性疾病的基本缺陷。预计在理解其他皮肤病的病理生物化学方面也会取得类似进展。本综述总结了我们关于糖胺聚糖的结构、合成、降解、大分子组织和可能功能的知识,糖胺聚糖是皮肤基质的重要组成部分。以黏多糖贮积症为例,说明了将疾病的临床症状与基本缺陷联系起来的困难。