Molecular analysis of the iduronate-2-sulfatase gene in Thai patients with Hunter syndrome. J Inherit Metab Dis 31 Suppl 2, S303–S311. Crossref , Medline , Google Scholar

2018-07-24

J Med Genet. 1999 Jan;36(1):21-7. [PubMed:9950361] Villani GR, Daniele A, Balzano N, Di Natale P: Expression of five iduronate-2-sulfatase site-directed mutations. 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.

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).

Enzymet ges som en infusion veckovis. Det finns risk för allergiska Patients with Hunter syndrome do not produce an enzyme called iduronate-2-sulfatase.

Test Information. This biochemical test is a quantitative measurement of iduronate-2-sulfatase enzyme activity and can be used as a 1st tier test for patients with a

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.

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.

2 4 6 månadersschema Iduronate 2 sulfatase.

Enzymes IDURONATE 2-SULFATASE; IDS · TEXT · ▽ Description · ▻ Cloning and Expression · ▻ Gene Structure · ▻ Mapping · ▻ Molecular Genetics · ▻ ALLELIC NX_P22304 - IDS - Iduronate 2-sulfatase - Function. Lysosomal enzyme involved in the degradation pathway of dermatan sulfate and heparan sulfate. Mucopolysaccharidosis II, (MPS II, Hunter syndrome) is an X-linked lysosomal storage disorder caused by the deficiency of iduronate sulfatase (IDS) enzyme Invitrogen Anti-Iduronate 2 Sulfatase Monoclonal (OTI1D9), Catalog # MA5- 25855. Tested in Western Blot (WB), Immunofluorescence (IF), Immunocytochemistry Iduronate 2-sulfatase (IDS) is a lysosomal exohydrolase belonging to the sulfatase family and is required for the degradation of the glycosaminoglycans heparan Test Information. This biochemical test is a quantitative measurement of iduronate-2-sulfatase enzyme activity and can be used as a 1st tier test for patients with a Alpha-L-iduronate sulfate sulfatase. Short name: Idursulfase.
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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.

2020 — Magyar · Română. Iduronat-2-sulfatas - Iduronate-2-sulfatase. Från Wikipedia, den fria encyklopedin. iduronat-2-sulfatas.
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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).

Institute for the Study of Inborn Errors of Metabolism, Faculty of Science, Pontificia Universidad Javeriana, Bogotá, Colombia. Idursulfase is a purified form of human iduronate-2-sulfatase, a lysosomal enzyme. Idursulfase is produced by recombinant DNA technology in a human cell line.

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Actr2, ARP2 actin-related protein 2, 8703, 131.47, 165.12, 121.77, 139.45, 1358. Actr3, ARP3 Ids, iduronate 2-sulfatase, 2315, 24.8, 25.31, 23.49, 24.53, 7023.

Alpha-L-iduronate sulfate sulfatase Short: Idursulfase. Cleaved into the following 2 chains. Iduronate 2-sulfatase 14 kDa chain. Iduronate 2-sulfatase 42 kDa chain. 2017-06-08 · 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 dermatan 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).