Mucopolysaccharides (also known as Glycosaminoglycans or GAGs) are long chains of sugar molecules that are essential for building the bones, cartilage, skin, tendons, and other tissues of the body. Normally, the human body continuously breaks down and builds mucopolysaccharides. There are several enzymes involved in breaking down each GAG and a deficiency or absence of any of the essential enzymes can cause the GAG to not be broken down completely and result in its accumulation in the tissues and organs in the body.
This accumulation of mucopolysaccharides is known as mucopolysaccharidosis (MPS). There are a number of inherited diseases that are caused by this accumulation of mucopolysaccharides, and they result in problems with an individual’s development. With each condition, mucopolysaccharides accumulate in the cells and tissues of the body because of a deficiency of a specific enzyme. The specific enzyme that is deficient or absent is what distinguishes one type of MPS from another.
In some MPS conditions, in addition to the GAG being stored in the body, some of the incompletely broken GAGs can leave the body via the urine. When too much GAG is stored, organs and tissues can be damaged or not function properly.
