Elaprase (API Idursulfas)

3,500.00200,000.00

The drug is used for the treatment of a rare inherited disease that affects only males- Hunter syndrome. Due to the inability to breakdown specific metabolic byproducts, they build up throughout the body which causes walking and breathing difficulties. Without treatment, the symptoms become more severe over time. The dose depends on the patient’s body weight (0.5mg/kg), and the drug is given every week through slow intravenous infusion.

Description

Buy Elaprase (API Idursulfas)

Pharmacodynamics

Buy Elaprase (API Idursulfas) is a purified shape of the lysosomal enzyme human iduronate-2-sulfatase of recombinant DNA beginning. it’s far designed to update the herbal enzyme, growing catabolism of positive amassed glycosaminoglycans (GAG), which abnormally collect in multiple tissue sorts in sufferers with mucopolysaccharidosis II (MPS-II, or Hunter syndrome). Buy Elaprase (API Idursulfas)

Mechanism of action

Hunter’s Syndrome is an X-related recessive disease due to insufficient tiers 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 steadily collect inside the lysosomes of a spread of cells, main to cellular engorgement, organomegaly, tissue destruction and organ device dysfunction. remedy of Hunter’s Syndrome sufferers with idursulfase gives exogenous enzyme for uptake into cellular lysosomes. concentrated on of idursulfase to the lysosome takes place by using endocytosis from the cell floor. Mannose-6-phosphate (M6P) residues on the oligosaccharide chains allow specific binding of the enzymes to the M6P receptors at the mobile surface, leading to mobile internalization of the enzyme, concentrated on to intracellular lysosomes and subsequent catabolism of amassed GAG.

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Additional information

Quantity

1vial (6mg), 3 vial (6mg), 5 vial (6mg), 10 vial (6mg), 100 vial (6mg)