Anti-IDUA Antibody Picoband®

SKU: A02281-2

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC,IF,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 2-5 μg/ml, Human, Mouse, Rat
Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: IDUA

Specificity:

Background: This gene encodes an enzyme that hydrolyzes the terminal alpha-L-iduronic acid residues of two glycosaminoglycans, dermatan sulfate and heparan sulfate. This hydrolysis is required for the lysosomal degradation of these glycosaminoglycans. Mutations in this gene that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I).

Immunogen: E.coli-derived human INSIG1 recombinant protein (Position: M1-R87).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Alif, N., Hess, K., Straczek, J., Sebbar, S., N'Bou, A., Nabet, P., Dousset, B. Mucopolysaccharidosis type I: characterization of a common mutation that causes Hurler syndrome in Moroccan subjects. Ann. Hum. Genet. 63: 9-16, 1999. 2. Aronovich, E. L., Pan, D., Whitley, C. B. Molecular genetic defect underlying alpha-L-iduronidase pseudodeficiency. Am. J. Hum. Genet. 58: 75-85, 1996. 3. Ashton, L. J., Brooks, D. A., McCourt, P. A. G., Muller, V. J., Clements, P. R., Hopwood, J. J. Immunoquantification and enzyme kinetics of alpha-L-iduronidase in cultured fibroblasts from normal controls and mucopolysaccharidosis type I patients. Am. J. Hum. Genet. 50: 787-794, 1992.

Uniprot ID: P35475

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross reactivity with other proteins.

Isotype: IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 73 kDa

Calculated Molecular Weight: 52588 MW

Gene ID: 3425

Protein Name: Alpha-L-iduronidase

Gene Full Name: alpha-L-iduronidase

Synonyms: IDUA; Alpha-L-iduronidase