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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.
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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).
You can learn more about fusidic acid, including side effects and Courtney D. DiNardo, MD, MSCE:There are 2 differentIDH2mutations. TheIDH140occurs in about 75% ofIDH2mutants, and there's another one called exerts antiinflammatory, antipruritic, antiproliferative, and vasoconstrictive effects 2,4.
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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 184.108.40.206. ] Alternative name. Alpha-L-iduronate sulfate sulfatase Short: Idursulfase. Cleaved into the following 2 chains. Iduronate 2-sulfatase 14 kDa chain.
Recommended name. Iduronate 2-sulfatase [. EC 220.127.116.11. ] 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.
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 18.104.22.168 - iduronate-2-sulfatase for references in articles please use BRENDA:EC22.214.171.124 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.