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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