Proteintech, 86499-1-RR, IDUA Recombinant monoclonal antibody

Size: 20ul / 100ul The IDUA (86499-1-RR) by Proteintech is a Recombinant antibody targeting IDUA in WB, ELISA applications with reactivity to human, mouse, rat samples 86499-1-RR targets IDUA in WB, ELISA applications and shows reactivity with human, mouse, rat samples. Tested Applications Positive WB detected in: HEK-293 cells, HeLa cells, LNCaP cells, mouse brain tissue, rat brain tissue, mouse kidney tissue, rat kidney tissue Recommended dilution Western Blot (WB): WB : 1:1000-1:4000 Background Information Iduronidase (L-iduronidase, alpha-L-iduronidase, laronidase) is an enzyme with the systematic name glycosaminoglycan alpha-L-iduronohydrolase. This enzyme catalyzes the hydrolysis of unsulfated alpha-L-iduronosidic linkages in dermatan sulfate. It is a glycoprotein enzyme found in the lysosomes of cells. It is involved in the degeneration of glycosaminoglycans such as dermatan sulfate and heparan sulfate. The enzyme acts by hydrolyzing the terminal alpha-L-iduronic acid residues of these molecules, degrading them (PMID: 4993544,30407). A deficiency in the IDUA protein is associated with mucopolysaccharidoses (MPS). MPS, a type of lysosomal storage disease, is typed I through VII. In this syndrome, glycosaminoglycans accumulate in the lysosomes and cause substantial disease in many different tissues of the body. IDUA mutations result in the MPS 1 phenotype, which is inherited in an autosomal recessive fashion. The defective alpha-L-iduronidase results in an accumulation of heparan and dermatan sulfate within phagocytes, endothelium, smooth muscle cells, neurons, and fibroblasts. Prenatal diagnosis of this enzyme deficiency is possible (PMID:8242073). Specification Tested Reactivity: human, mouse, rat Host / Isotype: Rabbit / IgG Class: Recombinant Type: Antibody Immunogen: CatNo: Ag30658 Product name: Recombinant human IDUA protein Source: e coli. -derived, PGEX-4T Tag: GST Domain: 441-555 aa of NM_000203 Sequence: YASDDTRAHPNRSVAVTLRLRGVPPGPGLVYVTRYLDNGLCSPDGEWRRLGRPVFPTAEQFRRMRAAEDPVAAAPRPLPAGGRLTLRPALRLPSLLLVHVCARPEKPPGQVTRLR Predict reactive species Full Name: iduronidase, alpha-L- Calculated Molecular Weight: 73 kDa Observed Molecular Weight: 73-75 kDa GenBank Accession Number: NM_000203 Gene Symbol: IDUA Gene ID (NCBI): 3425 Conjugate: Unconjugated Form: Liquid Purification Method: Protein A purification UNIPROT ID: P35475 Storage Buffer: PBS with 0.02% sodium azide and 50% glycerol, pH 7.3. Storage Conditions: Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20 o C storage. 20ul sizes contain 0.1% BSA.