Proteintech, 30006-1-AP, IDUA Polyclonal antibody

Size: 20ul / 150ul The IDUA (30006-1-AP) by Proteintech is a Polyclonal antibody targeting IDUA in WB, IHC, ELISA applications with reactivity to Human, Mouse, rat samples 30006-1-AP targets IDUA in WB, IHC, ELISA applications and shows reactivity with Human, Mouse, rat samples. Tested Applications Positive WB detected in: A549 cells, HEK-293 cells, LNCaP cells, mouse brain tissue, rat brain tissue Positive IHC detected in: mouse kidney tissueNote: suggested antigen retrieval withTE buffer pH 9.0;(*) Alternatively, antigen retrieval may be performed withcitrate buffer pH 6.0 Recommended dilution Western Blot (WB): WB : 1:500-1:3000 Immunohistochemistry (IHC): IHC : 1:50-1:500 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: Polyclonal 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 kDa GenBank Accession Number: NM_000203 Gene Symbol: IDUA Gene ID (NCBI): 3425 RRID: AB_2935498 Conjugate: Unconjugated Form: Liquid Purification Method: Antigen affinity 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.