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Anti-Bestrophin/BEST1 Antibody Picoband® Fluoro647 Conjugated

Product Specifications

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.

Synonyms

Bestrophin-1; TU15B; Vitelliform macular dystrophy protein 2; BEST1; VMD2

Gene Name

BEST1

Gene ID

7439

UniProt

O76090

Host

Rabbit

Reactivity

Human

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

A synthetic peptide corresponding to a sequence at the N-terminus of human Bestrophin, which shares 62.5% amino acid (aa) sequence identity with mouse Bestrophin.

Clonality

Polyclonal

Tissue Specificity

Predominantly expressed in the basolateral membrane of the retinal pigment epithelium.

Applications

Flow Cytometry

Field of Research

Cell Adhesion Proteins, ECM Proteins, Neuroscience, Sensory System, Visual System

Purification

Immunogen affinity purified.

Form

Liquid

Function

Forms calcium-sensitive chloride channels. Highly permeable to bicarbonate.

References & Citations

1. Entrez Gene: BEST1 bestrophin 1. 2. Johnson AA, Guziewicz KE, Lee CJ, Kalathur RC, Pulido JS, Marmorstein LY, Marmorstein AD (January 2017) . Bestrophin 1 and retinal disease. review. Progress in Retinal and Eye Research. 58: 45–69.

Storage Conditions

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

Calculated Molecular Weight

68 kDa

Specificity

No cross reactivity with other proteins.

Applications Notes

6

Gene Name Synonym

Bestrophin 1

Subcellular Location

Cell membrane.

Protein Name

Keratin, type I cytoskeletal 14

Isotype

Rabbit IgG

Contents

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.

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