Invitrogen™ Bestrophin 1 Polyclonal Antibody

Catalog No.	Quantity
PIPA578867	100 μg

Catalog No. PIPA578867 Supplier Invitrogen™ Supplier No. PA578867

Description

Rabbit Polyclonal Antibody

Reconstitute with 0.2 mL of distilled water to yield a concentration of 500 μg/mL. Positive Control - WB: human A549 whole cell, human SGC-7901 whole cell, human U20S whole cell. Flow: Hela cell, LOVO cell.

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, known as 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 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 a reflection of 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 reduces or abolishes Bestrophin protein mediated membrane current. The human Bestrophin gene encodes a 585 amino acid protein.

Specifications

• Antigen: Bestrophin 1
• Applications: Western Blot
• Classification: Polyclonal
• Concentration: 500 μg/mL
• Conjugate: Unconjugated
• Formulation: PBS with 4mg trehalose, 0.09mg NaCl, 0.2mg Na2PO4 and no preservative
• Gene: BEST1
• Gene Accession No.: O76090
• Gene Alias: ARB; BEST; BEST 1; Best disease; best macular dystrophy; BEST1; Best1V1Delta2; bestrophin; bestrophin 1; Bestrophin1; Bestrophin-1; BMD; Bmd1; LOW QUALITY PROTEIN: bestrophin-1; mBest1; RP50; TU15B; vitelliform macular dystrophy (Best disease, bestrophin); vitelliform macular dystrophy 2 homolog; vitelliform macular dystrophy protein 2; Vitelliform macular dystrophy protein 2 homolog; VMD 2; Vmd2
• Gene Symbols: BEST1
• Host Species: Rabbit
• Immunogen: A synthetic peptide corresponding to a sequence of human Bestrophin (RFIYRLALTEEQQLMFEKLTLYCD).
• Purification Method: Antigen affinity chromatography
• Quantity: 100 μg
• Regulatory Status: RUO
• Primary or Secondary: Primary
• Gene ID (Entrez): 7439
• Target Species: Human
• Content And Storage: -20°C
• Product Type: Antibody
• Form: Lyophilized
• Isotype: IgG