Enzyme levels may be normal in individuals receiving enzyme replacement therapy or who have undergone bone marrow transplant. Iduronate-2-sulfatase can also be deficient in individuals with multiple sulfatase deficiency.
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.
This antibody reacts with human, mouse, rat , pig samples. Cat.No. 66112-1-Ig. 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. Calnexin promotes the folding of mutant iduronate 2-sulfatase related to mucopolysaccharidosis type II. Biochem Biophys Res Commun 514 , 217–223. Crossref , Medline , Google Scholar 2006-04-01 Production and characterization of a human lysosomal recombinant iduronate‐2‐sulfatase produced in Pichia pastoris. Natalia Pimentel.
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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).
Brist på enzymet har påvisats vid Hunters av MG till startsidan Sök — Molecular basis of mucopolysaccharidosis type II: Mutations in the iduronate sulfatase gene. Hum Mut 1993; 2: 435-442. Hunter CA. Mucopolysaccharidosis Type II Observational MPS II is a rare X-linked recessive genetic disease caused by mutations in the iduronate-2-sulfatase gene (IDS).
Idursulfas (handelsnamn Elaprase) är ett läkemedel som används för att behandla Hunters sjukdom (också kallad Mucopolysaccharoidosis II).[1]
Iduronate 2-sulfatase 14 kDa chain. Iduronate 2-sulfatase 42 kDa chain.
Anti-Iduronate 2 sulfatase/SIDS antibody (ab85701) at 0.1 µg/ml + Human Liver lysate at 35 µg Predicted band size: 62 kDa Observed band size: 62 kDa. Immunohistochemistry (Formalin/PFA-fixed paraffin-embedded sections) - Anti-Iduronate 2 sulfatase/SIDS antibody (ab85701)
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.
Protein class i. Assigned HPA protein class(es) for the encoded protein (s). Detection of Human Iduronate 2-Sulfatase/IDS antibody by Western Blot. Catalog #.
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Cleaved into the following 2 chains: Iduronate 2-sulfatase 42 kDa chain. Iduronate 2-sulfatase 14 IDS. Gene description i. Full gene name according to HGNC. Iduronate 2- sulfatase. Protein class i.
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. Among 18 human sulfatase genes identified, about 1/3 are found in lysosomes and serve to remove sulfate from glycosaminoglycans (GAGs), glycopeptides and glycolipids
Our Iduronate 2-Sulfatase/IDS Antibodies can be used in a variety of model species: Canine, Human, Monkey, Mouse, Rat. Use the list below to choose the Iduronate 2-Sulfatase/IDS Antibody which is most appropriate for your research; you can click on each one to view full technical details, images, references, reviews and related products.
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2006-04-01
Cite This Product: Human IDS/Iduronate 2 Sulfatase ELISA Kit PicoKine™ (Boster Biological Technology, Pleasanton CA, USA, Catalog # EK1452) ELISA Validation This product was previously labelled as Iduronate 2 sulfatase .