Laronidase, Recombinant Human IDUA

Specification

• Cat#: THP-0132
• Product Name: Laronidase, Recombinant Human IDUA
• Description: Human recombinant alpha-L-iduronidase(IDUA) encodes 628a.a.(mature form) and is expressed in a CHO cell line. The product is a glycoprotein with a molecular weight of approximately 83 kD. The predicted amino acid sequence of the recombinant form, as well as the nucleotide sequence that encodes it, is identical to a polymorphic form of human a-L-iduronidase. It contains 6 N-linked oligosaccharide modification sites.
• Sequences: APHLVQVDAARALWPLRRFWRSTGFCPPLPHSQADQYVLSWDQQLNLAYVGAVPHRGIKQVRTHWLLELVTTRGSTGRGLSYNFTHLDGYLDLLRENQLLPGFELMGSASGHFTDFEDKQQVFEWKDLVSSLARRYIGRYGLAHVSKWNFETWNEPDHHDFDNVSMTMQGFLNYYDACSEGLRAASPALRLGGPGDSFHTPPRSPLSWGLLRHCHDGTNFFTGEAGVRLDYISLHRKGARSSISILEQEKVVAQQIRQLFPKFADTPIYNDEADPLVGWSLPQPWRADVTYAAMVVKVIAQHQNLLLANTTSAFPYALLSNDNAFLSYHPHPFAQRTLTARFQVNNTRPPHVQLLRKPVLTAMGLLALLDEEQLWAEVSQAGTVLDSNHTVGVLASAHRPQGPADAWRAAVLIYASDDTRAHPNRSVAVTLRLRGVPPGPGLVYVTRYLDNGLCSPDGEWRRLGRPVFPTAEQFRRMRAAEDPVAAAPRPLPAGGRLTLRPALRLPSLLLVHVCARPEKPPGQVTRLRALPLTQGQLVLVWSDEHVGSKCLWTYEIQFSQDGKAYTPVSRKPSTFNLFVFSPDTGAVSGSYRVRALDYWARPGPFSDPVPYLEVPVPRGPPSPGNP
• Species: Human
• Molecular Weight: 69899.4 Da
• Source: CHO
• Purity: >99% by SDS-Page and HPLC analysis
• Cas No: 210589-09-6
• Formula: C3160H4848N898O881S12
• Endotoxin Level: <0.001 EU per 1 μg of the protein by the LAL method
• Biological Activity: 100IU/ml

Pharmaceutical Information

• Pharmaceutical Application: Laronidase
• Applications: For the treatment of mucopolysaccharidosis
• Examples of Clinical Use: Mucopolysaccharidosis
• Pharmacodynamics: The product is used to treat mucopolysaccharide storage disorders (specifically mucopolysaccharidosis 1 or Hurlers syndrome) caused by deficiencies of alpha-L-iduronidase. Reduced or absent a-L-iduronidase activity results in the accumulation of the GAG substrates, dermatan sulfate and heparan sulfate, throughout the body and leads to widespread cellular, tissue, and organ dysfunction.
• Mechanism of action: Laronidase catalyses the hydrolysis of terminal alpha-L-iduronic acid residues of dermatan sulfate and heparin sulfate.
• Affected organisms: Humans and other mammals
• Targets: Target 1. Iduronic acid