Iduronate 2-Sulfatase/IDS: Products Sulfatases belong to a highly conserved family of enzymes that catalyze the hydrolysis of O- and N-sulfate esters from a variety of substrates.

This syndrome results from a deficiency of α- l-iduronate-2-sulfatase, an enzyme that hydrolyses sulfated iduronic acid residues in heparan sulfate and dermatan sulfate. Dermatan sulfate and heparan sulfate accumulate in the lysosomes of affected cells.

Assigned HPA protein class(es) for the encoded protein (s). Detection of Human Iduronate 2-Sulfatase/IDS antibody by Western Blot. Catalog #. Availability. Size Goat polyclonal Iduronate 2 sulfatase/SIDS antibody. Validated in WB, IHC and tested in Human.

As a member of the wwPDB, the RCSB PDB curates and annotates PDB data according to agreed upon standards. The RCSB PDB also provides a variety of tools and resources. Users can perform simple and advanced searches based on annotations relating to sequence, structure and function. These molecules are visualized, downloaded, and analyzed by users who range from students to specialized scientists. Iduronate-2-sulfatase (IDS) is a lysosomal sulfatase that prevents the accumulation within the brain of glycosoaminoglycans. However, IDS does not cross the blood−brain barrier (BBB).

Från Wikipedia, den fria encyklopedin. iduronat-2-sulfatas. Identifierare.

Hydrolysis of the 2-sulfate groups of the L-iduronate 2-sulfate units of dermatan sulfate, heparan sulfate and heparin. By similarity Manual assertion inferred from sequence similarity to i

2016-12-09 · DiNatale and Ronsisvalle (1981) identified 2 forms of iduronate sulfatase from human placenta and Bielicki et al. (1990) purified 2 major forms with molecular masses of 42 kD and 14 kD from human liver. Wilson et al. (1990) isolated and sequenced a 2.3-kb cDNA clone coding for the entire sequence of human IDS from an endothelial cell cDNA library.

Demonstration of deficient iduronate-2-sulfatase enzyme activity is considered the gold standard to confirm a diagnosis of Mucopolysaccharidosis II (MPS II), Hunter Syndrome. In addition, it can be used to clarify molecular findings in the IDS gene and to follow up abnormal newborn screening results. Turnaround Time . 14 days. CPT Code(s) 82657

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This message will disappear when all data is loaded. EC Tree 3 Hydrolases 3.1 Acting on ester bonds The present invention provides a highly glycosylated iduronate-2-sulfatase enzyme comprising an iduronate-2-sulfatase polypeptide with at least 5 kilodalton (kDa) more sugar than iduronate-2-sulfatase purified from a natural source, e.g. human liver. The present invention also provides an enzymatically active polypeptide fragment or variant of such a highly glycosylated iduronate-2-sulfatase. iduronate sulfatase: an enzyme required for the desulfation of 2-sulfate iduronate residues in heparan sulfate. It is also required in dermatan sulfate degradation; Hunter syndrome is associated with a deficiency of this enzyme. As a member of the wwPDB, the RCSB PDB curates and annotates PDB data according to agreed upon standards.
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Idursulfase is an enzyme that hydrolyzes the 2-sulfate esters of terminal iduronate sulfate residues from the glycosaminoglycans dermatan sulfate and heparan sulfate in the lysosomes of various cell types. Iduronate 2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this X-chromosome gene that result in enzymatic deficiency lead to the sex-linked mucopolysaccharidosis type II, also known as Hunter syndrome.

Hunter syndrome is the only MPS syndrome to exhibit X-linked recessive inheritance. The symptoms of Hunter syndrome are comparable to those of MPS I. Abstract Hunter syndrome is a rare but devastating childhood disease caused by mutations in the IDS gene encoding iduronate-2-sulfatase, a crucial enzyme in the lysosomal degradation pathway of The full iduronate-2-sulfatase sequence contains seven possible N-glycosylation sites, but the number used in the 42-kDa polypeptide is not known.
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As a member of the wwPDB, the RCSB PDB curates and annotates PDB data according to agreed upon standards. The RCSB PDB also provides a variety of tools and resources. Users can perform simple and advanced searches based on annotations relating to sequence, structure and function. These molecules are visualized, downloaded, and analyzed by users who range from students to specialized scientists.

Iduronate-2-sulfatase (IDS) is a lysosomal sulfatase that prevents the accumulation within the brain of glycosoaminoglycans. However, IDS does not cross the blood−brain barrier (BBB). To enable BBB transport, human IDS, minus its signal peptide, was fused to the carboxyl terminus of the heavy chain of a chimeric monoclonal antibody (mAb) to the human insulin receptor (HIR). The HIRMAb Iduronate 2 sulfatase antibody Mouse Monoclonal from Proteintech validated in Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF),Enzyme-linked Immunosorbent Assay (ELISA) applications. This antibody reacts with human, mouse, rat , pig samples. Cat.No. 66112-1-Ig.