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BEST1 / BEST / Bestrophin

bestrophin 1

BEST1 / BEST / Bestrophin is a member of the bestrophin gene family. This small gene family is characterized by proteins with a highly conserved N-terminus with four to six transmembrane domains. Bestrophins may form chloride ion channels or may regulate voltage-gated L-type calcium-ion channels. Bestrophins are generally believed to form calcium-activated chloride-ion channels in epithelial cells but they have also been shown to be highly permeable to bicarbonate ion transport in retinal tissue. Mutations in this gene are responsible for juvenile-onset vitelliform macular dystrophy (VMD2), also known as Best macular dystrophy, in addition to adult-onset vitelliform macular dystrophy (AVMD) and other retinopathies. Alternative splicing results in multiple variants encoding distinct isoforms.

Gene Name: bestrophin 1
Family/Subfamily: Ion channel , Bestrophin
Synonyms: BEST1, ARB, Best1V1Delta2, Bestrophin-1, BEST, Best disease, Bestrophin, Bestrophin 1, BMD, Hbest1, VMD2, RP50, TU15B
Target Sequences: NM_004183 NP_004174.1 O76090

Publications (10)

1
Bestrophin, the product of the Best vitelliform macular dystrophy gene (VMD2), localizes to the basolateral plasma membrane of the retinal pigment epithelium. Marmorstein AD, Marmorstein LY, Rayborn M, Wang X, Hollyfield JG, Petrukhin K. Proceedings of the National Academy of Sciences of the United States of America. 2000 97:12758-63. (ICC, WB, IP, IHC-Fr; Human, Pig) [PubMed:11050159] [PMC:PMC18837]
2
Bestrophin interacts physically and functionally with protein phosphatase 2A. Marmorstein LY, McLaughlin PJ, Stanton JB, Yan L, Crabb JW, Marmorstein AD. The Journal of biological chemistry. 2002 277:30591-7. (WB; Pig) [PubMed:12058047]
3
Derivation and comparative assessment of retinal pigment epithelium from human embryonic stem cells using transcriptomics. Klimanskaya I, Hipp J, Rezai KA, West M, Atala A, Lanza R. Cloning and stem cells. 2004 6:217-45. (ICC; Human) [PubMed:15671670]
4
Bestrophin gene mutations cause canine multifocal retinopathy: a novel animal model for best disease. Guziewicz KE, Zangerl B, Lindauer SJ, Mullins RF, Sandmeyer LS, Grahn BH, Stone EM, Acland GM, Aguirre GD. Investigative ophthalmology & visual science. 2007 48:1959-67. (ICC, WB; Dog) [PubMed:17460247] [PMC:PMC1931491]
5
Hemangioblastic derivatives from human induced pluripotent stem cells exhibit limited expansion and early senescence. Feng Q, Lu SJ, Klimanskaya I, Gomes I, Kim D, Chung Y, Honig GR, Kim KS, Lanza R. Stem cells (Dayton, Ohio). 2010 28:704-12. (ICC; Human) [PubMed:20155819]
6
Bestrophin 2 is expressed in human non-pigmented ciliary epithelium but not retinal pigment epithelium. Zhang Y, Patil RV, Marmorstein AD. Molecular vision. 2010 16:200-6. (WB; Human) [PubMed:20157619] [PMC:PMC2820107]
7
Equivalence of conventionally-derived and parthenote-derived human embryonic stem cells. Harness JV, Turovets NA, Seiler MJ, Nistor G, Altun G, Agapova LS, Ferguson D, Laurent LC, Loring JF, Keirstead HS. PloS one. 2011 6:e14499. (ICC, WB; Human) [PubMed:21249129] [PMC:PMC3017547]
8
Molecular consequences of BEST1 gene mutations in canine multifocal retinopathy predict functional implications for human bestrophinopathies. Guziewicz KE, Slavik J, Lindauer SJ, Aguirre GD, Zangerl B. Investigative ophthalmology & visual science. 2011 52:4497-505. (WB; Dog) [PubMed:21498618] [PMC:PMC3175949]
9
Generation of retinal pigment epithelial cells from human embryonic stem cell-derived spherical neural masses. Cho MS, Kim SJ, Ku SY, Park JH, Lee H, Yoo DH, Park UC, Song SA, Choi YM, Yu HG. Stem cell research. 2012 9:101-9. (ICC; Human) [PubMed:22683799]
10
The culture and maintenance of functional retinal pigment epithelial monolayers from adult human eye. Blenkinsop TA, Salero E, Stern JH, Temple S. Methods in molecular biology (Clifton, N.J.). 2013 945:45-65. [PubMed:23097100]
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Antibodies (30)
Proteins (7)
Over-Expression Lysate (2)
Primary (30)
Recombinant (5)
BEST1 / BEST / Bestrophin (37)
Human (29)
Mouse (10)
Rat (5)
Bovine (1)
IHC (9)
IHC-P (1)
WB (27)
Flo (4)
ELISA (3)
ICC (1)
IF (2)
Peptide-ELISA (1)
rabbit (24)
mouse (4)
rabbit-new zealand white (2)
IgG (13)
IgG1,k (2)
IgG2a (1)
monoclonal mc (3)
polyclonal pc (27)
1C2 (1)
APC (1)
APC Conjugated (1)
Biotin Conjugated (1)
Cy3 Conjugated (1)
Cy7 Conjugated (1)
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Unconjugated (21)
No (37)
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Human (6)
293T Cells (1)
E. coli (2)
HEK 293 Cells (2)
Wheat Germ Extract (2)
Purified (3)
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal (DY488) Antibody
Human
Flo
DY488 Conjugated
100 µg/$571
BEST1 / BEST / Bestrophin Antibody - Bestrophin Antibody (E6-6) - Western blot detection of Bestrophin (68 kDa) from human RPE cell lysate.
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BEST1 / BEST / Bestrophin Mouse anti-Human Monoclonal Antibody
Human
IF, WB
Unconjugated
0.1 ml/$386
BEST1 / BEST / Bestrophin Antibody - Immunofluorescence analysis of U2OS cells using BEST1 antibody. Blue: DAPI for nuclear staining.
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Mouse, Human
IF, WB
Unconjugated
20 µl/$275; 50 µl/$311; 100 µl/$389; 200 µl/$503
BEST1 / BEST / Bestrophin Antibody - Western blot - Anti-Bestrophin Picoband antibody
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Human
Flo, ICC, IHC, WB
Unconjugated
10 µg/$318; 100 µg/$470
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Human
ELISA, IHC, WB
Unconjugated
100 µg/$331
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Human
ELISA, IHC, WB
Unconjugated
100 µg/$331
BEST1 / BEST / Bestrophin Antibody - Immunohistochemistry of paraffin-embedded rat brain tissue.
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Mouse, Rat, Human
IHC, WB
Unconjugated
20 µl/$275; 50 µl/$311; 100 µl/$389; 200 µl/$503
BEST1 / BEST / Bestrophin Antibody - Immunohistochemical analysis of Bestrophin-1 staining in rat brain formalin fixed paraffin embedded tissue section. The section was pre-treated using heat mediated antigen retrieval with sodium citrate buffer (pH 6.0). The section was then incubated with the antibody at room temperature and detected using an HRP conjugated compact polymer system. DAB was used as the chromogen. The section was then counterstained with hematoxylin and mounted with DPX.
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Mouse, Rat, Human
IHC, IHC-P, WB
Unconjugated
100 µl/$321
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BEST1 / BEST / Bestrophin Rabbit-New Zealand White anti-Mouse Polyclonal Antibody
Mouse
IHC, WB
Unconjugated
100 µl/$393
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Human
IHC, WB
Unconjugated
50 µl/$356; 100 µl/$461
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Mouse, Rat, Human
IHC, WB
Unconjugated
60 µl/$331; 120 µl/$403; 200 µl/$529
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BEST1 / BEST / Bestrophin Mouse anti-Human Monoclonal (1C2) Antibody
Human
ELISA, WB
Unconjugated
100 µg/$423
BEST1 / BEST / Bestrophin Antibody - Western blot testing of human 1) A549, 2) SGC-7901 and 3) U-2 OS cell lysate with Bestrophin 1 antibody at 0.5ug/ml. Predicted molecular weight ~68 kDa.
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Human
Flo, WB
Unconjugated
100 µg/$575
Select
BEST1 / BEST / Bestrophin Rabbit-New Zealand White anti-Human Polyclonal Antibody
Human
IHC, WB
Unconjugated
100 µl/$393
BEST1 / BEST / Bestrophin Antibody - Western Blot; Sample: Human U87-MG cell lysate; Primary Ab: 1µg/ml Rabbit Anti-Human BEST1 Antibody Second Ab: 0.2µg/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Human
WB
Unconjugated
100 µl/$309; 200 µl/$351; 500 µl/$491; 1 ml/$561; 2 ml/$755; 5 ml/$1,225
BEST1 / BEST / Bestrophin Antibody - Western Blot; Sample: Human U87-MG cell lysate; Primary Ab: 1µg/ml Rabbit Anti-Human BEST1 Antibody Second Ab: 0.2µg/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal (HRP) Antibody
Human
WB
HRP Conjugated
100 µl/$338; 200 µl/$393; 500 µl/$575; 1 ml/$702; 2 ml/$939; 5 ml/$1,593
BEST1 / BEST / Bestrophin Antibody - Western Blot; Sample: Human U87-MG cell lysate; Primary Ab: 1µg/ml Rabbit Anti-Human BEST1 Antibody Second Ab: 0.2µg/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal (Biotin) Antibody
Human
WB
Biotin Conjugated
100 µl/$319; 200 µl/$365; 500 µl/$519; 1 ml/$596; 2 ml/$824; 5 ml/$1,348
BEST1 / BEST / Bestrophin Antibody - Western Blot; Sample: Human U87-MG cell lysate; Primary Ab: 1µg/ml Rabbit Anti-Human BEST1 Antibody Second Ab: 0.2µg/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal (FITC) Antibody
Human
WB
FITC Conjugated
100 µl/$348; 200 µl/$407; 500 µl/$605; 1 ml/$755; 2 ml/$993; 5 ml/$1,716
BEST1 / BEST / Bestrophin Antibody - Western Blot; Sample: Human U87-MG cell lysate; Primary Ab: 1µg/ml Rabbit Anti-Human BEST1 Antibody Second Ab: 0.2µg/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal (Cy3) Antibody
Human
WB
Cy3 Conjugated
100 µl/$407; 200 µl/$491; 500 µl/$850; 1 ml/$1,008; 2 ml/$1,308; 5 ml/$2,451
BEST1 / BEST / Bestrophin Antibody - Western Blot; Sample: Human U87-MG cell lysate; Primary Ab: 1µg/ml Rabbit Anti-Human BEST1 Antibody Second Ab: 0.2µg/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal (APC, Cy7) Antibody
Human
WB
APC, Cy7 Conjugated
100 µl/$505; 200 µl/$648; 500 µl/$1,152; 1 ml/$1,401; 2 ml/$1,961; 5 ml/$3,676
BEST1 / BEST / Bestrophin Antibody - Western Blot; Sample: Human U87-MG cell lysate; Primary Ab: 1µg/ml Rabbit Anti-Human BEST1 Antibody Second Ab: 0.2µg/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody
Select
BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal (APC) Antibody
Human
WB
APC Conjugated
100 µl/$407; 200 µl/$491; 500 µl/$850; 1 ml/$1,008; 2 ml/$1,308; 5 ml/$2,451
BEST1 / BEST / Bestrophin Antibody - Western Blot; Sample: Human U87-MG cell lysate; Primary Ab: 1µg/ml Rabbit Anti-Human BEST1 Antibody Second Ab: 0.2µg/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody
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BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal (PE) Antibody
Human
WB
PE Conjugated
100 µl/$407; 200 µl/$491; 500 µl/$850; 1 ml/$1,008; 2 ml/$1,308; 5 ml/$2,451
Select
BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Mouse, Bovine, Rat, Human
Peptide-ELISA, WB
Unconjugated
100 µl/$379; 200 µl/$421
Select
BEST1 / BEST / Bestrophin Mouse anti-Human Monoclonal Antibody
Human
Unconjugated
100 µg/$351
Select
BEST1 / BEST / Bestrophin Rabbit anti-Human Polyclonal Antibody
Mouse, Human
WB
Unconjugated
60 µl/$331; 120 µl/$403; 200 µl/$529
Viewing 1-25 of 37 product results



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For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).