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GeneBio Systems

Anti-ALADIN/AAAS Antibody Picoband®

Anti-ALADIN/AAAS Antibody Picoband®

SKU:A01336-3

Regular price €506,95 EUR
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Size: 100 μg

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

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Flow Cytometry (Fixed), 1-3 μg/1x106 cells, Human
ELISA, 0.1-0.5 μg/ml

Gene Name: AAAS

Specificity:

Background: The protein encoded by this gene is a member of the WD-repeat family of regulatory proteins and may be involved in normal development of the peripheral and central nervous system. The encoded protein is part of the nuclear pore complex and is anchored there by NDC1. Defects in this gene are a cause of achalasia-addisonianism-alacrima syndrome (AAAS), also called triple-A syndrome or Allgrove syndrome. Two transcript variants encoding different isoforms have been found for this gene.

Immunogen: E.coli-derived human ALADIN/AAAS recombinant protein (Position: R13-K466). Human ALADIN/AAAS shares 94.3% amino acid (aa) sequence identity with mouse ALADIN/AAAS.

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.Bitetto, G., Ronchi, D., Bonato, S., Pittaro, A., Compagnoni, G. M., Bordoni, A., Salani, S., Frattini, E., Lopez, G., Cribiu, F. M., Corti, S., Comi, G. P., Bresolin, N., Di Fonzo, A. Loss of the nucleoporin aladin in central nervous system and fibroblasts of Allgrove syndrome. Hum. Molec. Genet. 28: 3921-3927, 2019. 2. Chang, A. J., Kline, M. M., Currie, Y., Perez, M. O., Hartiala, J., Wijesuriya, H., Buchanan, T. A., Watanabe, R. M., Allayee, H. Allgrove syndrome in a Mexican American family is caused by an ancestral mutation derived from North Africa. (Letter) Clin. Genet. 73: 385-387, 2008. 3. Cronshaw, J. M., Matunis, M. J. The nuclear pore complex protein ALADIN is mislocalized in triple A syndrome. Proc. Nat. Acad. Sci. 100: 5823-5827, 2003.

Uniprot ID: Q9NRG9

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: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 60 kDa

Calculated Molecular Weight: 60 kDa

Gene ID: 8086

Protein Name: Aladin

Gene Full Name: aladin WD repeat nucleoporin

Synonyms: AAA; AAAS; AAASb; ADRACALA; ADRACALIN; ALADIN; GL003

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