Anticorps Recombinant de lapin anti-IDUA

IDUA Recombinant Antibody for WB, ELISA

Hôte / Isotype

Lapin / IgG

Réactivité testée

Humain, rat, souris

Applications

WB, ELISA

Conjugaison

Non conjugué

CloneNo.

251274F3

N° de cat : 86499-1-RR

Synonymes

Alpha-L-iduronidase, EC:3.2.1.76, MPS1

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Galerie de données de validation

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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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Applications testées

• Résultats positifs en WB: cellules HEK-293, cellules HeLa, cellules LNCaP, tissu cérébral de rat, tissu cérébral de souris, tissu rénal de rat, tissu rénal de souris

Dilution recommandée

• 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

Informations sur le produit

86499-1-RR cible IDUA dans les applications de WB, ELISA et montre une réactivité avec des échantillons Humain, rat, souris

• Réactivité: Humain, rat, souris
• Hôte / Isotype: Lapin / IgG
• Clonalité: Recombinant
• Type: Anticorps
• Immunogène: IDUA Protéine recombinante Ag30658
• Nom complet: iduronidase, alpha-L-
• Masse moléculaire calculée: 73 kDa
• Poids moléculaire observé: 73-75 kDa
• Numéro d’acquisition GenBank: NM_000203
• Symbole du gène: IDUA
• Identification du gène (NCBI): 3425
• Conjugaison: Non conjugué
• Forme: Liquide
• Méthode de purification: Purification par protéine A
• Tampon de stockage: PBS with 0.02% sodium azide and 50% glycerol
• Conditions de stockage: Stocker à -20°C. Stable pendant un an après l'expédition. L'aliquotage n'est pas nécessaire pour le stockage à -20^oC Les 20ul contiennent 0,1% de BSA.

Informations générales

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).

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