Cat# : THP-0178
|THP-0178||Idursulfase, Human iduronate-2-sulfatase(IDS)||April 02, 2023||100ug||$1,498||
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|Product Name:||Idursulfase, Human iduronate-2-sulfatase(IDS)|
|Description:||The product is a purified form of human iduronate-2-sulfatase(IDS), a lysosomal enzyme, expressed in a human cell line. The product 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. The product is a 525-amino acid glycoprotein with a molecular weight of approximately 76 KDa. The enzyme contains eight asparagine-linked glycosylation sites occupied by complex oligosaccharide structures. The enzyme activity of the product is dependent on the post-translational modification of a specific cysteine to formylglycine.|
|Molecular Weight:||76000.0 Da|
|Purity:||>99% by SDS-Page and HPLC analysis|
|Endotoxin Level:||<0.001 EU per 1 μg of the peptide by the LAL method|
|Applications:||For the treatment of Hunter syndrome in adults and children ages 5 and older.|
|Examples of Clinical Use:||Hunter syndrome|
|Pharmacodynamics:||The product is a purified form of the lysosomal enzyme human iduronate-2-sulfatase of recombinant DNA origin. It is designed to replace the natural enzyme, increasing catabolism of certain accumulated glycosaminoglycans (GAG), which abnormally accumulate in multiple tissue types in patients with mucopolysaccharidosis II (MPS-II, or Hunter syndrome).|
|Mechanism of action:||Hunter's Syndrome is an X-linked recessive disease caused by insufficient levels of the lysosomal enzyme iduronate-2-sulfatase. This enzyme cleaves the terminal 2-O-sulfate moieties from the glycosaminoglycans (GAG) dermatan sulfate and heparan sulfate. Due to the missing or defective iduronate-2-sulfatase enzyme in patients with Hunter's Syndrome, GAG progressively accumulate in the lysosomes of a variety of cells, leading to cellular engorgement, organomegaly, tissue destruction and organ system dysfunction. Treatment of Hunter's Syndrome patients with idursulfase provides exogenous enzyme for uptake into cellular lysosomes. Targeting of idursulfase to the lysosome occurs by endocytosis from the cell surface. Mannose-6-phosphate (M6P) residues on the oligosaccharide chains allow specific binding of the enzymes to the M6P receptors on the cell surface, leading to cellular internalization of the enzyme, targeting to intracellular lysosomes and subsequent catabolism of accumulated GAG.|
|Affected organisms:||Humans and other mammals|
|Targets:||Target 1. Dermatan sulfate; Target 2. Heparan sulfate; Target 3. Perilipin-3|
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