What are the mucopolysaccharidoses?
What are the mucopolysaccharidoses?
The mucopolysaccharidoses are a group of lysosomal storage diseases characterized by the accumulation of excess mucopolysaccharides (GAGs). Skeletal deformities arise from the accrual of GAGs in the chondrocytes, resulting in abnormal ossifïcation. Examples include Hurler's syndrome, Hunter's syndrome, Morquio's syndrome, Sanfilippo's syndrome, Maroteaux-Lamy syndrome, and other forms, depending on the site of the enzymatic defect.