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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. Hydrolysis of the 2-sulfate groups of the L-iduronate 2-sulfate units of dermatan sulfate, heparan sulfate and heparin. Hunter syndrome is caused by a deficiency of the lysosomal enzyme iduronate-2-sulfatase (I2S). The lack of this enzyme causes heparan sulfate and dermatan sulfate to accumulate in all body tissues. 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.

Cleaved into the following 2 chains. Iduronate 2-sulfatase 14 kDa chain. Iduronate 2-sulfatase 42 kDa chain. The IDS gene provides instructions for producing an enzyme called iduronate 2-sulfatase (I2S), which is essential for the breakdown of large sugar molecules called glycosaminoglycans (GAGs).

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Invitrogen Anti-Iduronate 2 Sulfatase Monoclonal (OTI4G2), Catalog # MA5-25866. Tested in Western Blot (WB), Immunofluorescence (IF), Immunocytochemistry (ICC), Immunohistochemistry (Paraffin) (IHC (P)) and Flow Cytometry (Flow) applications. This antibody reacts with Canine, Human, Mouse, Non-human primate, Rat samples.

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IDS is one of the sulfatase enzymes required for lysosomal degradation of glycosaminoglycans. Mutant proteins linked to diseases are often prone to misfolding. 2020-10-02 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 2021-03-02 Iduronate 2 Sulfatase Antibodies Iduronate 2 Sulfatase Antibodies Iduronate-2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this X-chromosome gene that result 2017-06-08 This product was previously labelled as Iduronate 2 sulfatase The Life Science industry has been in the grips of a reproducibility crisis for a number of years. Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for … This is a TMB colorimetric sandwich ELISA kit with short assay time and fast experiment set up. Iduronate 2-Sulfatase/IDS tissue specificity: Liver, kidney, lung, and placenta.

Iduronate 2 Sulfatase Lysosomal Degradation of Heparin and Heparan Sulfate. Peter J. Meikle, Iduronate-2-sulfatase is an exo Cardiological Aspects of Systemic Disease. Robert F. English, José A. Ettedgui, in Paediatric Cardiology (Third I. A recombinant form of iduronate-2-sulfatase 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.
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Iduronate 2-sulfatase [. EC 3.1.6.13. ] Alternative name. Alpha-L-iduronate sulfate sulfatase Short: Idursulfase. Cleaved into the following 2 chains. Iduronate 2-sulfatase 14 kDa chain.

2016-12-09 Recommended name. Iduronate 2-sulfatase [. EC 3.1.6.13. ] Alternative name. Alpha-L-iduronate sulfate sulfatase Short: Idursulfase. Cleaved into the following 2 chains.
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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. MPS II, resulting from a deficiency of iduronate-2-sulfatase, is the only x-linked MPS, with most mutations being restricted to individual families (75–77). The presence of a pseudodeficiency allele may cause reduced activity of iduronate-2-sulfatase in the artificial substrate used in this assay. Enzyme levels may be normal in individuals receiving enzyme replacement therapy or who have undergone bone marrow transplant.

2020 — Magyar · Română. Iduronat-2-sulfatas - Iduronate-2-sulfatase. Från Wikipedia, den fria encyklopedin.
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Iduronatsulfatas Svensk MeSH

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. Recombinant Human Iduronate 2 sulfatase/IDS Protein (Met1-Pro550) 10337-H08H with a fusion His Tag, is expressed in HEK293 Cells. With high purity, high biological activity, high stability, and other superior features, you can use this Human Iduronate 2 sulfatase/IDS protein for relevant bioassay and related research. 102100008356 Iduronate 2-sulfatase Human genes 0.000 title claims description 377 238000000746 purification Methods 0.000 title description 20 101710019405 BN863_22000 Proteins 0.000 title 1 Information on EC 3.1.6.13 - iduronate-2-sulfatase for references in articles please use BRENDA:EC3.1.6.13 The purpose of this study is to determine if one year of therapy with iduronate-2-sulfatase enzyme replacement therapy, at a dose of 0.5mg/kg, weekly or every other week, is safe, and results in clinically meaningful improvement in multiple organ function, compared with a placebo group.

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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.

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(CAS nr) 7664-93-9. (EC nr) 231-639-5. (Index nr) "Anaerobic sulfatase maturase AslB from Escherichia coli activates human recombinant iduronate-2-sulfate sulfatase (IDS) and N-acetylgalactosamine-6-sulfate the lysosomal enzyme iduronate-2-sulfatase. Hunters syndrom är en X-kromosomkopplad sjukdom orsakad av otillräckliga nivåer av det lysosomala enzymet Iduronate-2-sulfatase. ▫ TKT UK Ltd. ▫ Treatment of Mucopolysaccharidosis type II (Hunter Syndrome). ▫ 05.09.2001.