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Anti-Bestrophin Antibody (Biotin)

FabGennix
BEST1.3-BIOTIN
Overview
Name: Anti-Bestrophin Antibody (Biotin)
Description: Rabbit polyclonal antibody to Bestrophin (Biotin)
Applications: CM, ELISA, IF, IHC, IP, WB
Dilutions: Confocal Microscopy: 1:100; DB: 1:10,000; ELISA: 1:10,000; Immunocytochemistry: 1:100; Immunofluorescence: 1:100; Immunohistochemistry: 1:100; Immunoprecipitation: 1:200; Western Blot: 1:473
Reactivity: Human
Host: Rabbit
Clonality: Polyclonal
Isotype: IgG
Conjugate: Biotin
Concentration: 0.75 µg/µl in antibody stabilization buffer
Storage: -20⁰C for long term storage

Target (Information from UniProt)
Function: Forms calcium-sensitive chloride channels. Highly permeable to bicarbonate.
Tissue Specificity: Predominantly expressed in the basolateral membrane of the retinal pigment epithelium.
Involvement in Disease: Macular dystrophy, vitelliform, 2: An autosomal dominant form of macular degeneration that usually begins in childhood or adolescence. VMD2 is characterized by typical 'egg-yolk' macular lesions due to abnormal accumulation of lipofuscin within and beneath the retinal pigment epithelium cells. Progression of the disease leads to destruction of the retinal pigment epithelium and vision loss.

Retinitis pigmentosa 50: A retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.

Bestrophinopathy, autosomal recessive: A retinopathy characterized by loss of central vision, an absent electro-oculogram light rise, and electroretinogram anomalies.

Vitreoretinochoroidopathy, autosomal dominant: A disorder characterized by vitreoretinochoroidal dystrophy. The clinical presentation is variable. VRCP may be associated with cataract, nanophthalmos, microcornea, shallow anterior chamber, and glaucoma.
Sequence Similarities: Belongs to the bestrophin family.
Post-Translational Modification: Phosphorylated by PP2A.
Cellular Location: Cell membrane. Basolateral cell membrane.
UniProt: O76090
Synonyms: ARB Antibody
BEST Antibody
BEST 1 Antibody
Best disease Antibody
Best macular dystrophy Antibody
BEST1 Antibody
Best1V1Delta2 Antibody
BEST1_HUMAN Antibody
Bestrophin 1 Antibody
Bestrophin-1 Antibody
Bestrophin1 Antibody
BMD Antibody
mBest1 Antibody
RP50 Antibody
TU15B Antibody
Vitelliform macular dystrophy Antibody
Vitelliform macular dystrophy 2 Antibody
Vitelliform macular dystrophy protein 2 Antibody
VMD 2 Antibody
VMD2 Antibody

Product Code - BEST1.3-BIOTIN
Size Price Quantity
100µg £475

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