2006-04-01

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.

Turnaround Time . 14 days. CPT Code(s) 82657 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. 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.

Iduronate 2-sulfatase

enzymet iduronate-2sulfatase och produceras genom DNA teknologi i en mänsklig cell linje. Enzymet ges som en infusion veckovis. Det finns risk för allergiska Patients with Hunter syndrome do not produce an enzyme called iduronate-2-sulfatase. Last Update: 2017-04-26. Usage Frequency: 3. Quality: Excellent Mucopolysaccharidosis type II or Hunter syndrome is a rare lysosomal storage disorder caused by a deficiency in the enzyme iduronate-2-sulfatase (IDS).

Immunogen corresponding to synthetic peptide.

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. 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.

Mucopolysaccharidosis type III: MedlinePlus Genetics image. Mucopolysaccharidosis type II Osteoimmunology in mucopolysaccharidoses type I, II, VI and Die Heparitinsulfat-Mucopolysaccharidose (Sanfilippo PDF) BMN 250, a fusion of lysosomal Iduronate 2-sulfatase (IDS) is a sulfatase enzyme associated with Hunter syndrome. Iduronate-2-sulfatase is an exo -sulfatase that hydrolyzes the C2-sulfate ester bond from nonreducing terminal α-L-iduronic acid residues in HS (Fig. 1), as well as DS. This enzyme has been purified 500,000-fold to homogeneity from human liver (73).

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.

14 days. CPT Code(s) 82657 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. 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. Users can perform simple and advanced searches based on annotations relating to sequence, structure and function.

Iduronate 2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. 2021-03-02 · Extensive iduronate 2-sulfatase (Hunter syndrome) (IDS) gene deletions were identified in four mucopolysaccharidosis type II (MPSII) patients. Two new mutations were discovered: p.K236N (c.708G>C) and p.Q80K (c.238C>A) which resulted in a severe phenotype and early death of Muccopolysaccharridosis Type II patients from Bulgaria and Macedonia. 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 Hunter syndrome is an X-linked recessive disease caused by deficiency of the lysosomal enzyme iduronate-2-sulfatase.
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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. 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.

Iduronate 2-sulfatase [. EC 3.1.6.13. ] Alternative name.
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Dvs patienter med iduronate-2-sulfatas brist enzymet iduronate-2sulfatase och produceras genom DNA teknologi i en mänsklig cell linje. Enzymet ges som en

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.

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The report on Global Iduronate 2 Sulfatase Market offers in-depth analysis on market trends, drivers, restraints, opportunities etc. Along with qualitative information, this report include the quantitative analysis of various segments in terms of market share, growth, opportunity analysis, market value, etc. for the forecast years.

antibody. Seroprotektionsfrekvens. Antibody.

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 …

Iduronate-2-sulfatase is an exo -sulfatase that hydrolyzes the C2-sulfate ester bond from nonreducing terminal α-L-iduronic acid residues in HS (Fig. 1), as well as DS. This enzyme has been purified 500,000-fold to homogeneity from human liver (73). Iduronate 2-sulfatase is a sulfatase enzyme that catalyze the hydrolysis of sulfate ester bonds from a wide variety of substrates, including steroids, carbohydrates and proteins.

Vi är stolta över att lista förkortningen av IDS i den största databasen av förkortningar och akronymer. Följande bild visar en av definitionerna för IDS på engelska: Iduronate 2-Sulfatase. The recombinant fusion protein SHP631 consists of a chimeric monoclonal antibody binding to human insulin receptor and iduronate-2-sulfatase (I2S). This product is being developed as an enzyme replacement therapy to treat cognitive symptoms of Hunter’s syndrome. iduronate sulfatase: an enzyme required for the desulfation of 2-sulfate iduronate residues in heparan sulfate.