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MPS type II, more commonly referred to as Hunter syndrome, is a form of MPS disorder. As with the other types of MPS, GAGs build up in cells throughout the body. In MPS II specifically, this GAG buildup is caused by an enzyme called iduronate-2-sulfatase (I2S) being deficient or missing altogether. Hunter syndrome is also unique from the other MPS types as it almost exclusively affects males and is estimated to affect 1 in 162,000 live male births.