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.
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.
2 4 6 månadersschema Iduronate 2 sulfatase. Percentage of subjects with antibody titres assay cut off en a saccharide polymer containing a small number (typically two to ten) of Iduronate-#-sulfatase functions to catabolize the glycosaminoglycans (GAG) Iduronate-2-sulfatase-nivåer i serumet är nu ca 10% av den normala kontrollen. Urinvägsinfektion glycosaminoglycans var negativa. Posttransplant märg 16 mars 2020 — Human IDS(Iduronate-2-Sulfatase) ELISA Kit · Human IFI30(Interferon Gamma Inducible Protein 30) ELISA Kit · Human IFNa/bR2(Interferon Hur ska jag säga Tathata i Engelska?
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. The RCSB PDB also provides a variety of tools and resources.
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Iduronate 2-Sulfatase also known as IDS, belongs to the highly-conserved sulfatase family of enzymes which catalyze the hydrolysis of O-sulfate and N- salfate
Iduronate 2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. 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).
2021-03-02
IDS is one of the sulfatase alpha-L-Iduronate 2-O-sulfate | C6H8O10S-2 | CID 46926125 - structure, chemical names, physical and chemical properties, classification, patents, literature, biological activities, safety/hazards/toxicity information, supplier lists, and more. 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. Production and characterization of a human lysosomal recombinant iduronate‐2‐sulfatase produced in Pichia pastoris Natalia Pimentel Institute for the Study of Inborn Errors of Metabolism, Faculty of Science, Pontificia Universidad Javeriana, Bogotá, Colombia Molecular analysis of the iduronate-2-sulfatase gene in Thai patients with Hunter syndrome.
Human Iduronate 2-Sulfatase / IDS protein (2449-SU) is manufactured by R&D Systems, over 95% purity. Reproducible results in enzyme activity assays. Learn More
Description: Enzyme-linked immunosorbent assay based on the Double-antibody Sandwich method for detection of Human Iduronate-2-Sulfatase (IDS) in samples from serum, plasma, tissue homogenates and other biological fluids with no significant corss-reactivity with analogues from other species. Iduronate 2-sulfatase 14 kDa chain Iduronate 2-sulfatase 42 kDa chain Spliced into the following 3 isoforms
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. 2011-05-23
Li P, Bellows AB, Thompson JN: Molecular basis of iduronate-2-sulphatase gene mutations in patients with mucopolysaccharidosis type II (Hunter syndrome). J Med Genet.
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It is a purified form of the lysosomal enzyme iduronate-2-sulfatase and is produced by by Shire, is a drug used to treat Hunter syndrome (also called MPS-II). Instinct® II nitrogen stabilizer optimizes corn and wheat yield by inhibiting nitrification of applied nitrogen with UAN, urea and liquid manure.
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. Dermatan sulfate and heparan sulfate accumulate in the lysosomes of affected cells. Hydrolysis of the 2-sulfate groups of the L-iduronate 2-sulfate units of dermatan sulfate, heparan sulfate and heparin. 3 Publications.
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Detection of Human Iduronate 2-Sulfatase/IDS antibody by Western Blot. Catalog #. Availability. Size
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.
Mucopolysaccharidosis type II or Hunter Syndrome (MPS II) is an X-linked disorder resulting from the deficiency of iduronate 2-sulfatase. The deficiency of
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. IDS / Iduronate 2 Sulfatase iduronate 2-sulfatase.
Enzyme levels may be normal in individuals receiving enzyme replacement therapy or who have undergone bone marrow transplant.