This disease is inherited in an x-linked recessive manner which results in a predominantly male disease.
Hunter syndrome is a mucopolysaccharidosis, meaning it is a disorder caused by the absence or malfunctioning of lysosomal enzymes, leading to an inability to break down glycosaminoglycans. Hunter syndrome is also referred to as Mucopolysaccharidosis II (MPS II).
Iduronate sulfatase is the enzyme deficient in Hunter's disease, which results in abnormal carbohydrate breakdown in the lysosome.
This is a glycosaminoglycan which accumulates in Hunter's disease due to the defect in iduronate sulfatase.
This is another glycosaminoglycan which accumulates in Hunter's disease due to the defect in iduronate sulfatase.
Unlike hurler's disease, hunter's disease does not have accumulation of glycosaminoglycans in the cornea and does not have corneal clouding.
Those with Hunter's syndrome are characterized by having aggressive behavior, hyperactivity, restlessness and other behavioral issues.
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