Thermo Fisher Scientific Bestrophin 1 (extracellular) Polyclonal Antibody

Applications

Product Specifications

Product Specific Information

Product is shipped at room temperature as a lyophilized powder and should be stored at -20°C upon receipt.
For reconstitution, add 50 µL of deionized water.

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.
This material appears as a yellow mass resembling an egg yolk that later darkens and becomes irregular in color, a process known as “scrambling the egg.”
Best disease is often associated with mutations in the Bestrophin gene, which encodes a protein localized to the basolateral plasma membrane of RPE cells and forms calcium-sensitive chloride channels.
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.