IDUA Rekombinanter Antikörper

IDUA Rekombinant Antikörper für WB, ELISA

Wirt / Isotyp

Kaninchen / IgG

Getestete Reaktivität

human, Maus, Ratte

Anwendung

WB, ELISA

Konjugation

Unkonjugiert

CloneNo.

251274F3

Kat-Nr. : 86499-1-RR

Synonyme

Alpha-L-iduronidase, EC:3.2.1.76, MPS1

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  WB analysis using 86499-1-RR

  Various lysates were subjected to SDS PAGE followed by western blot with 86499-1-RR (IDUA antibody) at dilution of 1:2000 incubated at room temperature for 1.5 hours.

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  Affinity and Kinetic Characterization of 86499-1-RR

  Biolayer interferometry (BLl) kinetic assays of 86499-1-RR against Human IDUA were performed. The affinity constant is 2.20 nM.

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Geprüfte Anwendungen

• Erfolgreiche Detektion in WB: HEK-293-Zellen, HeLa-Zellen, LNCaP-Zellen, Maushirngewebe, Mausnierengewebe, Rattenhirngewebe, Rattennierengewebe

Empfohlene Verdünnung

• Western Blot (WB): WB : 1:1000-1:4000

It is recommended that this reagent should be titrated in each testing system to obtain optimal results.

Sample-dependent, check data in validation data gallery

Produktinformation

86499-1-RR bindet in WB, ELISA IDUA und zeigt Reaktivität mit human, Maus, Ratten

• Getestete Reaktivität: human, Maus, Ratte
• Wirt / Isotyp: Kaninchen / IgG
• Klonalität: Rekombinant
• Typ: Antikörper
• Immunogen: IDUA fusion protein Ag30658
• Vollständiger Name: iduronidase, alpha-L-
• Berechnetes Molekulargewicht: 73 kDa
• Beobachtetes Molekulargewicht: 73-75 kDa
• GenBank-Zugangsnummer: NM_000203
• Gene symbol: IDUA
• Gene ID (NCBI): 3425
• Konjugation: Unkonjugiert
• Form: Liquid
• Reinigungsmethode: Protein-A-Reinigung
• Lagerungspuffer: PBS with 0.02% sodium azide and 50% glycerol
• Lagerungsbedingungen: Bei -20°C lagern. Nach dem Versand ein Jahr lang stabil Aliquotieren ist bei -20^oC Lagerung nicht notwendig. 20ul Größen enthalten 0,1% BSA.

Hintergrundinformationen

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).