Thermo Fisher Scientific Bestrophin Polyclonal Antibody

Applications and Tested Dilutions

Product Specifications

Target Information

The retinal pigment epithelium (RPE) and choroid represent a differentiated system of the eye that sustains normal retinal health and function. Best vitelliform macular dystrophy (Best disease) is an early-onset autosomal dominant condition characterized by accumulation of lipofuscin-like material within and beneath the RPE, leading to progressive central vision loss. The lipofuscin-like material appears as a yellow mass resembling egg yolk that later becomes darker and irregular in color, a process known as “scrambling the egg.”

Best disease is frequently associated with mutations in the Bestrophin gene, which encodes a protein containing 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 at the Bestrophin locus reduce or abolish Bestrophin-mediated membrane current. 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.