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Causes
By Mayo Clinic staffAs with all types of MPS, Hunter syndrome is caused when a person lacks a specific enzyme his or her body needs to break down glycosaminoglycans — molecules formed from long chains of complex carbohydrates.
In unaffected people, these enzymes are found in cell components called lysosomes. Lysosomes break down the complex carbohydrates into nutrients, such as proteins and simpler molecules so that your body can use them at the cellular level. These nutrients help your body build bone, cartilage, tendons, corneas, skin and connective tissue, and are also found in the fluid that lubricates your joints. MPS disorders are also referred to as lysosomal storage disorders.
The lysosomes use enzymes to break down glycosaminoglycans, as part of the body's normal recycling and renewal process. In a person with Hunter syndrome or other form of MPS, these enzymes either are missing or don't work correctly.
These breaking-down tasks involve 11 different enzymes, and the particular enzyme that is missing or malfunctioning largely determines the type of MPS disorder. As a result of these enzyme malfunctions, undigested glycosaminoglycans collect in the cells, blood and connective tissues, causing permanent and progressive damage. In the case of Hunter syndrome, the missing or malfunctioning enzyme is called iduronate-2-sulfatase.
