Products

Idursulfase (TP-019CL)

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. Idursulfase is a 525-amino acid glycoprotein with a molecular weight of approximately 76 kilodaltons. The enzyme contains eight asparagine-linked glycosylation sites occupied by complex oligosaccharide structures. The enzyme activity of idursulfase is dependent on the post-translational modification of a specific cysteine to formylglycine.      

Specifications
Classify
Protein therapeutics replacing a protein that is deficient or abnormal: Metabolic enzyme deficiencies
Therapeutic
Idursulphase (iduronate-2-sulphatase)
Trade Name
Elaprase
Function
Cleaves the terminal 2-O-sulphate moieties from the GAGs dermatan sulphate and heparan sulphate, thereby allowing their digestion and preventing GAG accumulation
Examples of Clinical Use
Mucopolysaccharidosis ii (Hunter syndrome)
References
Synonyms
Alpha-L-iduronate sulfate sulfatase, Iduronate 2-sulfatase

For lab research use only, not for diagnostic, therapeutic or any in vivo human use.

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