Thermo Fisher Scientific Bestrophin 1 Polyclonal Antibody

Applications

Western Blot (WB)

• Tested Dilution: 1:300–1:2,000

Immunohistochemistry (IHC)

• Tested Dilution: 1:300–1:2,000

Miscellaneous PubMed (Misc)

• View 4 publications

Product Specifications

Product Specific Information

• Reconstitute in 100 µL of sterile water.
• Centrifuge to remove any insoluble material.
• Specificity: Bestrophin 1.

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.
This lipofuscin-like material appears as a yellow mass like 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.