Which disease is classified as a mucopolysaccharide storage disease?

Hurler's syndrome is classified as a mucopolysaccharide storage disease, which is a type of genetic disorder caused by the accumulation of glycosaminoglycans (previously referred to as mucopolysaccharides) due to enzyme deficiencies. In Hurler's syndrome, there is a deficiency in the enzyme alpha-L-iduronidase, which leads to the buildup of specific mucopolysaccharides in the body, causing various symptoms that affect multiple systems, including growth delays, skeletal abnormalities, and cognitive decline.

This disease is part of a broader group of disorders known as lysosomal storage diseases, where specific enzymes required for the breakdown of complex molecules are deficient. The accumulation of undigested molecules can lead to cellular dysfunction and a wide array of clinical manifestations.

In contrast, the other options refer to different types of storage diseases: Pompe's disease is a glycogen storage disease caused by a deficiency in the enzyme that breaks down glycogen, while von Gierke disease and glycogen storage diseases focus primarily on the metabolism of glycogen rather than the metabolism of mucopolysaccharides. These distinctions highlight why Hurler's syndrome is uniquely categorized under mucopolysaccharide storage diseases.