Anti-Bestrophin-1/Best1 Antibody Picoband®

SKU: A01434-2

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: Mouse,Rat

Applications: WB,ELISA

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

Gene Name: Best1

Specificity:

Background: Bestrophin-1 (Best1) is a protein that, in humans, is encoded by the BEST1 gene. This gene encodes a member of the bestrophin gene family. This small gene family is characterized by proteins with a highly conserved N-terminus with four to six transmembrane domains. Bestrophins may form chloride ion channels or may regulate voltage-gated L-type calcium-ion channels. Bestrophins are generally believed to form calcium-activated chloride-ion channels in epithelial cells but they have also been shown to be highly permeable to bicarbonate ion transport in retinal tissue. Mutations in this gene are responsible for juvenile-onset vitelliform macular dystrophy (VMD2), also known as Best macular dystrophy, in addition to adult-onset vitelliform macular dystrophy (AVMD) and other retinopathies. Alternative splicing results in multiple variants encoding distinct isoforms.

Immunogen: E.coli-derived mouse Bestrophin-1/Best1 recombinant protein (Position: V9-E551). Mouse Best1 shares 64.3% amino acid (aa) sequence identity with human Best1.

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. Allikmets, R., Seddon, J. M., Bernstein, P. S., Hutchinson, A., Atkinson, A., Sharma, S., Gerrard, B., Li, W., Metzker, M. L., Wadelius, C., Caskey, C. T., Dean, M., Petrukhin, K. Evaluation of the Best disease gene in patients with age-related macular degeneration and other maculopathies. Hum. Genet. 104: 449-453, 1999. 2. Bakall, B., Marknell, T., Ingvast, S., Koisti, M. J., Sandgren, O., Li, W., Bergen, A. A. B., Andreasson, S., Rosenberg, T., Petrukhin, K., Wadelius, C. The mutation spectrum of the bestrophin protein--functional implications. Hum. Genet. 104: 383-389, 1999. 3. Boon, C. J. F., Klevering, B. J., den Hollander, A. I., Zonneveld, M. N., Theelen, T., Cremers, F. P. M., Hoyng, C. B. Clinical and genetic heterogeneity in multifocal vitelliform dystrophy. Arch. Ophthal. 125: 1100-1106, 2007.

Uniprot ID: O88870

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: 56,60 kDa

Calculated Molecular Weight: 52588 MW

Gene ID: 24115

Protein Name: Bestrophin-1

Gene Full Name: bestrophin 1

Synonyms: ARB; BEST; BEST1; bestrophin 1; Bestrophin-1; BMD; TU15B; VMD2