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Invitrogen™ Bestrophin 1 Polyclonal Antibody
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Catalog No. PIPA534709
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PIPA534709 100 μL
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Catalog No. PIPA534709 Supplier Invitrogen™ Supplier No. PA534709
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Rabbit Polyclonal Antibody

Recommended positive controls: NT2D1, PC-3, SK-N-SH. Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen Bestrophin 1
Applications Western Blot
Classification Polyclonal
Concentration 1 mg/mL
Conjugate Unconjugated
Formulation PBS with 1% BSA, 20% glycerol and 0.01% thimerosal; pH 7
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 Recombinant protein encompassing a sequence within the center region of human Bestrophin 1. The exact sequence is proprietary.
Purification Method Antigen affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 7439
Target Species Human
Content And Storage Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Product Type Antibody
Form Liquid
Isotype IgG
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WARNING: Cancer - www.P65Warnings.ca.gov
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