Elaprase (Shire Pharmaceuticals) - General Information
Elaprase (Shire Pharmaceuticals) is a purified form of human iduronate-2-sulfatase, a lysosomal enzyme. Elaprase (Shire Pharmaceuticals) is produced by recombinant DNA technology in a human cell line. Elaprase (Shire Pharmaceuticals) 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. Elaprase (Shire Pharmaceuticals) 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.
Pharmacology of Elaprase (Shire Pharmaceuticals)
Elaprase (Shire Pharmaceuticals) 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).
Elaprase (Shire Pharmaceuticals) for patients
Elaprase (Shire Pharmaceuticals) Interactions
Elaprase (Shire Pharmaceuticals) Contraindications
Additional information about Elaprase (Shire Pharmaceuticals)
Elaprase (Shire Pharmaceuticals) Indication: For the treatment of Hunter syndrome in adults and children ages 5 and older.
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. 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.
Drug Interactions: Not Available
Food Interactions: Not Available
Generic Name: Idursulfase
Synonyms: Not Available
Drug Category: Enzyme Replacement Agents
Drug Type: Biotech; Approved
Absorption: Not Available
Toxicity (Overdose): There is no experience with overdosage of Idursulfase in humans. Single intravenous doses of idursulfase up to 20 mg/kg were not lethal in male rats and cynomolgus monkeys (approximately 6.5 and 13 times, respectively, of the recommended human dose based on body surface area) and there were no clinical signs of toxicity.
Protein Binding: Not Available
Biotransformation: Not Available
Half Life: 44 ± 19 minutes
Dosage Forms of Elaprase (Shire Pharmaceuticals): Solution Intravenous drip
Chemical IUPAC Name: Not Available
Chemical Formula: C2654H4000N688O774S14
Idursulfase on Wikipedia: https://en.wikipedia.org/wiki/Idursulfase
Organisms Affected: Humans and other mammals