Recombinant Human IDUA therapeutic protein

• Cat.No.: IDUA-P025H
• Product Overview: Recombinant Human alpha-L-iduronidase, 628 residues (mature form), produced by recombinant DNA technology in a Chinese hamster ovary cell line. Laronidase 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, are identical to a polymorphic form of human a-L-iduronidase. It contains 6 N-linked oligosaccharide modification sites.

Specification

• Description: This gene encodes an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Mutations in this gene that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I). The expression product is the active ingredient of Aldurazyme.
• Source: CHO
• Species: Human
• Molecular Mass: 69.9 kDa
• AA Sequence: APHLVQVDAARALWPLRRFWRSTGF CPPLPHSQADQYVLSWDQQLNLAYV GAVPHRGIKQVRTHWLLELVT TRGSTGRGLSYNFTHLDGYLDLLRE NQLLPGFELMGSASGHFTDFEDKQQ VFEWKDLVSSLARRYIGRYGL AHVSKWNFETWNEPDHHDFDNVSMT MQGFLNYYDACSEGLRAASPALRLG GPGDSFHTPPRSPLSWGLLRH CHDGTNFFTGEAGVRLDYISLHRKG ARSSISILEQEKVVAQQIRQLFPKF ADTPIYNDEADPLVGWSLPQP WRADVTYAAMVVKVIAQHQNLLLAN TTSAFPYALLSNDNAFLSYHPHPFA QRTLTARFQVNNTRPPHVQLL RKPVLTAMGLLALLDEEQLWAEVSQ AGTVLDSNHTVGVLASAHRPQGPAD AWRAAVLIYASDDTRAH PNRSVAVTLRLRGVPPGPGLVYVTR YLDNGLCSPDGEWRRLGRPVFPTAE QFRRMRAAEDPVAAAPRPLPA GGRLTLRPALRLPSLLLVHVCARPE KPPGQVTRLRALPLTQGQLVLVWSD EHVGSKCLWTYEIQFSQDGKA YTPVSRKPSTFNLFVFSPDTGAVSG SYRVRALDYWARPGPFSDPVPYLEV PVPRGPPSPGNP
• Endotoxin: < 1.0 EU per μg of the protein
• Purity: >95%
• Storage: Can be stored at +4 centigrade short term (1-2weeks). For long term storage, aliquot and store at -20 centigrade or -70 centigrade. Avoidrepeated freezing and thawing cycles.

Gene Information

• Gene Name: IDUA iduronidase, alpha-L- [ Homo sapiens ]
• Official Symbol: IDUA
• Synonyms: IDUA; iduronidase, alpha-L-; alpha-L-iduronidase; MPS1; IDA
• Gene ID: 3425
• mRNA Refseq: NM_000203
• Protein Refseq: NP_000194
• MIM: 252800
• UniProt ID: P35475
• Chromosome Location: 4p16.3
• Pathway: Dermatan sulfate degradation, organism-specific biosystem; Dermatan sulfate degradation, conserved biosystem; Glycosaminoglycan degradation, organism-specific biosystem; Glycosaminoglycan degradation, conserved biosystem; Heparan sulfate degradation, organism-specific biosystem; Heparan sulfate degradation, conserved biosystem; Lysosome, organism-specific biosystem
• Function: L-iduronidase activity; cation binding