Part:BBa_K5499002
Iduronate-2-sulfatase (IDS)
Iduronate-2-sulfatase (IDS) is a lysosomal enzyme that splits the sulfate group in the C-2 positions from iduronic acid residues present in the mucopolysaccharides dermatan sulfate and heparan sulfate.
Usage and Biology
A Deficiency in this enzyme results in the accumulation of these glycosaminoglycans in the lysosomes and subsequently leads to the development of Hunter syndrome(or mucopolysaccharidosis type 2 ) in humans. The disorder is inherited as an X-linked recessive disease and has a broad spectrum of clinical phenotypes ranging from mild to severe forms. This heterogeneity has been postulated to reflect the presence of different mutations in the IDS gene.
Sequence and Features
- 10COMPATIBLE WITH RFC[10]
- 12COMPATIBLE WITH RFC[12]
- 21COMPATIBLE WITH RFC[21]
- 23COMPATIBLE WITH RFC[23]
- 25COMPATIBLE WITH RFC[25]
- 1000COMPATIBLE WITH RFC[1000]
profile
Name: Iduronate-2-sulfatase (IDS)
Base Pairs:1650 bp
Origin: Homo sapiens, synthetic
Properties: a kind of lysosomal enzyme that can degrade glycosaminoglycans(GAGs)
None |