Tag Jürgen W. Spranger

Mucopolysaccharidoses

Mucopolysaccharidoses are hereditary, progressive diseases caused by mutations of genes coding for lysosomal enzymes needed to degrade glycosaminoglycans (acid mucopolysaccharides). Glycosaminoglycans (GAGs) are long-chain complex carbohydrates composed of uronic acids, amino sugars, and neutral sugars. The major GAGs are chondroitin-4-sulfate,…