Rb1636-211110-WS

A synthetic peptide from c-terminal region of human Bestrophin 1 conjugated to an immunogenic carrier protein was used as the antigen.

TU15B, Vitelliform macular dystrophy protein 2, BEST1, VMD2

FUNCTION: Forms calcium-sensitive chloride channels. Highly permeable to bicarbonate.
Subcellular location: Cell membrane; Multi-pass membrane protein. Basolateral cell membrane
Tissue specificity: Predominantly expressed in the basolateral membrane of the retinal pigment epithelium.
Defects in BEST1 are the cause of vitelliform macular dystrophy type 2 (VMD2) also known as Best macular dystrophy (BMD). VMD2 is 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.
Defects in BEST1 are the cause of retinitis pigmentosa type 50 (RP50).
Defects in BEST1 are a cause of adult-onset vitelliform macular dystrophy (AVMD).
Defects in BEST1 are the cause of bestrophinopathy autosomal recessive (ARB).
Defects in BEST1 are the cause of vitreoretinochoroidopathy autosomal dominant (ADVIRC).

Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.

12 months after reconstitution

This item will be shipped to you at ambient temperature in a lyophilised form.

1. Marquardt A, et al. Hum. Mol. Genet. 7:1517-1525(1998)
2. Tsunenari T, et al. J. Biol. Chem. 278:41114-41125(2003)
3. Sun H, et al. Proc. Natl. Acad. Sci. U.S.A. 99:4008-4013(2002)
4. Caldwell G.M, et al. Genomics 58:98-101(1999)
5. Boon C.J.F, et al. Retina 29:835-847(2009)

For research use only

100 µl

Unconjugated antibody

NZ white rabbit

Whole serum

Polyclonal

Polyclonal, whole serum

IHC, WB (confirmed by recombinant protein). A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.

Specific for Bestrophin 1.

Human. Other species not yet tested.

Lyophilised

Reconstitute in 100 µl of sterile water. Centrifuge to remove any insoluble material.

Control peptide is available at $120 per 50 μg. Please enquire sales@osenses.com

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