Mucopolysaccharidosis type I is a rare condition in which the body lacks or does not have enough of an enzyme required to break down long chains of sugar molecules. These molecular chains are known as glycosaminoglycans (formerly called mucopolysaccharides). Consequently, the molecules accumulate in various body places, causing multiple health issues.
People with Mucopolysaccharidosis type I do not produce the enzyme lysosomal alpha-L-iduronidase. This enzyme aids in the breakdown of glycosaminoglycans, which are long chains of sugar molecules. These molecules are present throughout the body, most notably in mucus and fluid surrounding the joints.
Without the enzyme, glycosaminoglycans accumulate and harm organs such as the heart. The severity of the symptoms might vary from minor to severe. Moreover, the moderate variant attenuates MPS I, whereas the intense version is severe Mucopolysaccharidosis type I.