{"product_id":"proteintech-30006-1-ap","title":"Proteintech, 30006-1-AP, IDUA Polyclonal antibody","description":"Size: 20ul \/ 150ul\nThe IDUA (30006-1-AP) by Proteintech is a Polyclonal antibody targeting IDUA in WB, IHC, ELISA applications with reactivity to Human, Mouse, rat samples\n30006-1-AP targets IDUA in WB, IHC, ELISA applications and shows reactivity with Human, Mouse, rat samples.\n\u003cb\u003eTested Applications\u003c\/b\u003e\nPositive WB detected in: A549 cells, HEK-293 cells,  LNCaP cells,  mouse brain tissue,  rat brain tissue\nPositive 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\n\u003cb\u003eRecommended dilution\u003c\/b\u003e\nWestern Blot (WB): WB : 1:500-1:3000\nImmunohistochemistry (IHC): IHC : 1:50-1:500\n\u003cb\u003eBackground Information\u003c\/b\u003e\nIduronidase (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).\n\u003cb\u003eSpecification\u003c\/b\u003e\nTested Reactivity: Human, Mouse, rat\nHost \/ Isotype: Rabbit \/ IgG\nClass: Polyclonal\nType: Antibody\nImmunogen: 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\nFull Name: iduronidase, alpha-L-\nCalculated Molecular Weight: 73 kDa\nObserved Molecular Weight: 73 kDa\nGenBank Accession Number: NM_000203\nGene Symbol: IDUA\nGene ID (NCBI): 3425\nRRID: AB_2935498\nConjugate: Unconjugated\nForm: Liquid\nPurification Method: Antigen affinity purification\nUNIPROT ID: P35475\nStorage Buffer: PBS with 0.02% sodium azide and 50% glycerol, pH 7.3.\nStorage 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.","brand":"Proteintech","offers":[{"title":"Default Title","offer_id":46887675789481,"sku":"30006-1-AP","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/ar\/products\/proteintech-30006-1-ap","provider":"Iright","version":"1.0","type":"link"}