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Target intelligence / Profile preview
Iduronate 2-sulfatase (I2S)
- Target
- I2S
- Molecular classification
- Enzyme, Sulfatase
01
Overview
Iduronate 2-sulfatase (I2S) is a lysosomal enzyme essential for the degradation of glycosaminoglycans (GAGs), specifically heparan sulfate and dermatan sulfate. Deficiency or dysfunction of I2S due to mutations in the IDS gene leads to mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome.
- Other names
- L-idurono sulfate sulfataseiduronide-2-sulfate sulfatasesulfoiduronate sulfohydrolaseL-iduronosulfatase
02
Mechanism of action
Hydrolyzes C-2 sulfates from L-iduronic acid residues in heparan sulfate and dermatan sulfate.
03
Biological functions
Glycosaminoglycan catabolismLysosomal degradation pathways
04
Disease associations
Mucopolysaccharidosis type IIHunter syndrome
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