Anti-BBS1 Antibody Picoband®

SKU: A03072-2

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,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Mouse, Rat
ELISA, 0.1-0.5 μg/ml, Human

Gene Name: BBS1

Specificity:

Background: Bardet–Biedl syndrome 1 protein is a protein that in humans is encoded by the BBS1 gene. Mutations in this gene have been observed in patients with the major form (type 1) of Bardet-Biedl syndrome. The encoded protein may play a role in eye, limb, cardiac and reproductive system development.

Immunogen: E.coli-derived human ATF5 recombinant protein (Position: L11-R275). Human ATF5 shares 86.5% and 87.2% amino acid (aa) sequence identity with mouse and rat ATF5, respectively.

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. Badano, J. L., Ansley, S. J., Leitch, C. C., Lewis, R. A., Lupski, J. R., Katsanis, N. Identification of a novel Bardet-Biedl syndrome protein, BBS7, that shares structural features with BBS1 and BBS2. Am. J. Hum. Genet. 72: 650-658, 2003. 2. Badano, J. L., Kim, J. C., Hoskins, B. E., Lewis, R. A., Ansley, S. J., Cutler, D. J., Castellan, C., Beales, P. L., Leroux, M. R., Katsanis, N. Heterozygous mutations in BBS1, BBS2 and BBS6 have a potential epistatic effect on Bardet-Biedl patients with two mutations at a second BBS locus. Hum. Molec. Genet. 12: 1651-1659, 2003. 3. Beales, P. L., Badano, J. L., Ross, A. J., Ansley, S. J., Hoskins, B. E., Kirsten, B., Mein, C. A., Froguel, P., Scambler, P. J., Lewis, R. A., Lupski, J. R., Katsanis, N. Genetic interaction of BBS1 mutations with alleles at other BBS loci can result in non-mendelian Bardet-Biedl syndrome. Am. J. Hum. Genet. 72: 1187-1199, 2003.

Uniprot ID: Q8NFJ9

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: 65 kDa

Calculated Molecular Weight: 65 kDa

Gene ID: 582

Protein Name: BBSome complex member BBS1

Gene Full Name: Bardet-Biedl syndrome 1

Synonyms: Bardet Biedl syndrome 1, BBS1, BBS2 like protein 2, BBS2L2