Abstract: Certain embodiments provide a method of providing added peripheral IDS activity to a subject with Hunter syndrome, comprising administering to the subject a therapeutically effective dose of a pharmaceutical composition comprising a protein comprising an IDS amino acid sequence and a TfR binding domain. The added peripheral IDS activity can be measured by comparing levels of keratan sulfate (KS) in the subject after administration of the pharmaceutical composition relative to a baseline level. Certain embodiments also provide methods of providing a clinical benefit to a subject with Hunter syndrome and improving treatment in a patient having non-neuronopathic Hunter syndrome, as well as methods and treatment regimens for resolving infusion related reactions (IRRs).

Abstract: Certain embodiments provide a method of treating Hunter syndrome in a subject in need thereof, comprising administering to the subject a therapeutically effective dose of a pharmaceutical composition comprising an ETV:IDS protein.