ABSTRACT
A significant component of all tissues is the complex polysaccharides known as glycosaminoglycans (GAGs). In the past the term “acid mucopolysaccharide” was used to designate these extracellular macromolecules containing a preponderance of carbohydrate. As this nomenclature was confusing and lacked precision, the GAGs became the preferred designation (117). A vast body of information about GAGs now exists, and an understanding of the mucopolysaccharidoses (MPSs) requires an appreciation of the basic structure of the GAGs and proteoglycans (PGs).
