Thermo Fisher Scientific Bestrophin Polyclonal Antibody

Applications and Tested Dilution

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 in which accumulation of lipofuscin-like material within and beneath the RPE leads to progressive loss of central vision.
The lipofuscin-like material in the macular area appears as a yellow mass resembling the yolk of an egg 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 and localizes to the basolateral plasma membrane of RPE cells.
Human Bestrophin forms oligomeric chloride channels that are 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.

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