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Bestrophin Polyclonal Antibody, APC Conjugated

Product Specifications

Background

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. 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. Bestrophin Bestrophin 2, Bestrophin 3, and Bestrophin 4 are transmembrane proteins that contain a high percentage of aromatic residues, a conserved RFP (Arg-Phe-Pro) motif and they function as anion channels.

Synonyms

BEST 1; BEST1; BEST-1; BEST; Best macular dystrophy; BEST1; BEST1_HUMAN; Bestrophin 1; Bestrophin-1; Bestrophin1; BMD; mBest1; TU15B; Vitellorm macular dystrophy 2; Vitellorm macular dystrophy; Vitellorm macular dystrophy protein 2; VMD 2; VMD2.

Gene ID

5068

Cellular Locus

Cell membrane

Host

Rabbit

Cross Reactivity

Human, Others

Immunogen

251-350/585

Target

Bestrophin

Clonality

Polyclonal

Isotype

IgG

Conjugation

APC

Source

KLH conjugated synthetic peptide derived from human Bestrophin

Applications

IF (IHC-P), IF (IHC-F), IF (ICC)

Purification

Purified by Protein A.

Excitation Emission

650nm/660nm

Concentration

1µg/µl

Dilution

IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)

Buffer

Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Modification

Unmodified

Storage Conditions

Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Gene ID URL

5068

Predicted Cross Reactivity

Mouse, Rat, Dog, Cow, Pig, Horse, Rabbit

Curated Selection

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