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.