Active Recombinant Human IDUA, His-tagged

• Cat.No.: IDUA-27H
• Product Overview: Recombinant human IDUA (Ala26­Pro653), fused with 10*his tag at C-terminal, was expressed in Mouse NSO Cells, 71 kDa .

Specification

• Cat. No.: IDUA-27H
• Description: α-L-Iduronidase encoded by the IDUA gene is an important enzyme required for the lysosomal degradation of glycosaminoglycans (GAGs). It hydrolyzes the non-reducing terminal α-L-iduronic acid residues in GAGs including dermatan sulfate and heparan sulfate. Mutations in IDUA that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I). MPS I causes progressive cellular, tissue and organ damage, and several clinical studies using enzyme replacement therapy have shown promising benefit.
• Source: Mammalian cells
• Species: Human
• Form: Supplied as a 0.2 μm filtered solution in Sodium Acetate and NaCl.
• Purity: > 95% as determined by SDS-PAGE
• Activity: Measured by its ability to cleave a fluorogenic substrate, 4­Methylumbelliferyl α­L­iduronide. The specific activity is >7,500 pmol/min/µg.
• EndotoxinLevel: <1.0 EU per 1 μg of the protein by the LAL method
• Storage: 6 months from date of receipt, ­20 to ­70 °C as supplied; 3 months, ­20 to ­70 °C under sterile conditions after opening.
• OfficialSymbol: IDUA

Gene Information

• Gene Name: IDUA iduronidase, alpha-L- [ Homo sapiens ]
• Synonyms: IDUA;iduronidase, alpha-L-; IDA; MPS1; Alpha-L-iduronidase; EC 3.2.1.76; OTTHUMP000002173092; OTTHUMP00000112666; α-L-iduronidase precursor
• Gene ID: 3425
• mRNA Refseq: NM_000203
• Protein Refseq: NP_000194
• MIM: 252800
• UniProt ID: P35475
• Chromosome Location: 4p16.3
• Pathway: Dermatan sulfatedegradation; Dermatan sulfate degradation; Glycosaminoglycan degradation; Glycosaminoglycan degradation; Heparan sulfate degradation; Heparan sulfatedegradation; Lysosome; Metabolic pathways
• Function: L-iduronidaseactivity; cation binding