Thermo Fisher Scientific Bestrophin 1 Polyclonal Antibody

Applications and Tested Dilution

Product Specifications

Product Specific Information

The antibody was affinity-purified from rabbit antiserum by affinity chromatography using an epitope-specific immunogen. The purity is greater than 95% as determined by SDS-PAGE.

Target Information

The retinal pigment epithelium (RPE) and choroid form a differentiated system in the eye that supports normal retinal health and function.
Best vitelliform macular dystrophy (Best disease) is an early-onset autosomal dominant disorder characterized by the accumulation of lipofuscin-like material within and beneath the RPE, leading to progressive central vision loss.
This material appears as a yellow mass resembling an egg yolk that later darkens and becomes irregular in color, a process referred to as “scrambling the egg.”
Best disease is often associated with mutations in the Bestrophin gene, which encodes a protein with four putative transmembrane domains localized to the basolateral plasma membrane of RPE cells.
Human Bestrophin forms oligomeric chloride channels sensitive to intracellular calcium. Missense mutations in the Bestrophin locus can reduce or abolish Bestrophin-mediated membrane currents. The human Bestrophin gene encodes a 585 amino acid protein.

For Research Use Only.
Not for use in diagnostic procedures. Not for resale without express authorization.