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

Background: Iduronate 2-Sulfatase/IDS As a member of the sulfatase family, IDS is required for the lysosomal degradation of the glycosaminoglycans (GAG) heparan sulfate and dermatan sulfate (2, 3). It hydrolyzes the 2-sulfate group of the L-iduronate 2-sulfate units of the GAG.

Natalia Pimentel. 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. Idursulfase is an enzyme that hydrolyzes the 2-sulfate esters of terminal iduronate sulfate residues from the glycosaminoglycans dermatan sulfate and heparan sulfate in the lysosomes of various cell types. Iduronate 2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate.

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. 2016-12-09 Recommended name. Iduronate 2-sulfatase [. EC 3.1.6.13.

Abcam is leading the way in addressing this with our range of recombinant monoclonal antibodies and knockout edited cell lines for gold-standard validation. 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 (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

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 The IDS gene encodes for an enzyme called iduronate-2-sulfatase (I2S).

Abstract : Mucopolysaccharidosis type II or Hunter syndrome is a rare lysosomal storage disorder caused by a deficiency in the enzyme iduronate-2-sulfatase

2016-12-09 · DiNatale and Ronsisvalle (1981) identified 2 forms of iduronate sulfatase from human placenta and Bielicki et al. (1990) purified 2 major forms with molecular masses of 42 kD and 14 kD from human liver. 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.

Iduronate 2-sulfatase [. EC 3.1.6.13. ] Alternative name. Alpha-L-iduronate sulfate sulfatase Short: Idursulfase. Cleaved into the following 2 chains. Iduronate 2-sulfatase 14 kDa chain.
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Hunter syndrome is the only MPS syndrome to exhibit X-linked recessive inheritance.

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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.

Catalog #. Availability. Size Goat polyclonal Iduronate 2 sulfatase/SIDS antibody. Validated in WB, IHC and tested in Human. Immunogen corresponding to synthetic peptide.