product summary
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company name :
Novus Biologicals
other brands :
IMGENEX
product type :
antibody
product name :
Bestrophin 1 Antibody (E6-6) - BSA Free
catalog :
NB300-164
quantity :
0.1 ml (also 0.025 ml)
price :
479 USD
clonality :
monoclonal
host :
mouse
conjugate :
Alexa Fluor 405
clone name :
E6-6
reactivity :
primate, human, mouse, rat, dogs, pigs
application :
western blot, immunohistochemistry, immunocytochemistry, immunoprecipitation, immunohistochemistry - paraffin section, immunohistochemistry - frozen section, proximity ligation assay
more info or order :
citations: 50
Published Application/Species/Sample/DilutionReference
  • immunocytochemistry; human; 1:100; fig 3f
Li J, Qiu C, Wei Y, Yuan W, Liu J, Cui W, et al. Human Amniotic Epithelial Stem Cell-Derived Retinal Pigment Epithelium Cells Repair Retinal Degeneration. Front Cell Dev Biol. 2021;9:737242 pubmed publisher
  • immunohistochemistry; human; 1:200; fig 4
Geng Z, Walsh P, Truong V, Hill C, Ebeling M, Kapphahn R, et al. Generation of retinal pigmented epithelium from iPSCs derived from the conjunctiva of donors with and without age related macular degeneration. PLoS ONE. 2017;12:e0173575 pubmed publisher
  • immunocytochemistry; dogs; 1:500; loading ...; fig 1b
  • western blot; dogs; 1:4000; loading ...; fig 1a
Uggenti C, Briant K, Streit A, Thomson S, Koay Y, Baines R, et al. Restoration of mutant bestrophin-1 expression, localisation and function in a polarised epithelial cell model. Dis Model Mech. 2016;9:1317-1328 pubmed
  • immunocytochemistry; human
Bertolotti E, Neri A, Camparini M, Macaluso C, Marigo V. Stem cells as source for retinal pigment epithelium transplantation. Prog Retin Eye Res. 2014;42:130-44 pubmed publisher
  • immunohistochemistry - paraffin section; human
  • immunocytochemistry; human
Sharon D, Al Hamdani S, Engelsberg K, Mizrahi Meissonnier L, Obolensky A, Banin E, et al. Ocular phenotype analysis of a family with biallelic mutations in the BEST1 gene. Am J Ophthalmol. 2014;157:697-709.e1-2 pubmed publisher
  • immunocytochemistry; human
Cho M, Kim S, Ku S, Park J, Lee H, Yoo D, et al. Generation of retinal pigment epithelial cells from human embryonic stem cell-derived spherical neural masses. Stem Cell Res. 2012;9:101-9 pubmed publisher
  • immunocytochemistry; human
Krohne T, Westenskow P, Kurihara T, Friedlander D, Lehmann M, Dorsey A, et al. Generation of retinal pigment epithelial cells from small molecules and OCT4 reprogrammed human induced pluripotent stem cells. Stem Cells Transl Med. 2012;1:96-109 pubmed
  • western blot; dogs
Guziewicz K, Slavik J, Lindauer S, Aguirre G, Zangerl B. Molecular consequences of BEST1 gene mutations in canine multifocal retinopathy predict functional implications for human bestrophinopathies. Invest Ophthalmol Vis Sci. 2011;52:4497-505 pubmed publisher
  • immunocytochemistry; human
  • western blot; human
Harness J, Turovets N, Seiler M, Nistor G, Altun G, Agapova L, et al. Equivalence of conventionally-derived and parthenote-derived human embryonic stem cells. PLoS ONE. 2011;6:e14499 pubmed publisher
  • western blot; human
Zhang Y, Patil R, Marmorstein A. Bestrophin 2 is expressed in human non-pigmented ciliary epithelium but not retinal pigment epithelium. Mol Vis. 2010;16:200-6 pubmed
  • immunocytochemistry; human
Feng Q, Lu S, Klimanskaya I, Gomes I, Kim D, Chung Y, et al. Hemangioblastic derivatives from human induced pluripotent stem cells exhibit limited expansion and early senescence. Stem Cells. 2010;28:704-12 pubmed publisher
  • western blot; human
Buchholz D, Hikita S, Rowland T, Friedrich A, Hinman C, Johnson L, et al. Derivation of functional retinal pigmented epithelium from induced pluripotent stem cells. Stem Cells. 2009;27:2427-34 pubmed publisher
  • western blot; human
Lu B, Malcuit C, Wang S, Girman S, Francis P, Lemieux L, et al. Long-term safety and function of RPE from human embryonic stem cells in preclinical models of macular degeneration. Stem Cells. 2009;27:2126-35 pubmed publisher
  • immunocytochemistry; dogs
  • western blot; dogs
Guziewicz K, Zangerl B, Lindauer S, Mullins R, Sandmeyer L, Grahn B, et al. Bestrophin gene mutations cause canine multifocal retinopathy: a novel animal model for best disease. Invest Ophthalmol Vis Sci. 2007;48:1959-67 pubmed
  • immunocytochemistry; human
Klimanskaya I, Hipp J, Rezai K, West M, Atala A, Lanza R. Derivation and comparative assessment of retinal pigment epithelium from human embryonic stem cells using transcriptomics. Cloning Stem Cells. 2004;6:217-45 pubmed
  • western blot; pigs
Marmorstein L, McLaughlin P, Stanton J, Yan L, Crabb J, Marmorstein A. Bestrophin interacts physically and functionally with protein phosphatase 2A. J Biol Chem. 2002;277:30591-7 pubmed
  • immunohistochemistry - frozen section; human
  • immunoprecipitation; human
  • immunocytochemistry; human
  • western blot; human
  • immunohistochemistry - frozen section; pigs
  • immunoprecipitation; pigs
  • immunocytochemistry; pigs
  • western blot; pigs
  • immunohistochemistry - frozen section; primate
  • immunoprecipitation; primate
  • immunocytochemistry; primate
  • western blot; primate
Marmorstein A, Marmorstein L, Rayborn M, Wang X, Hollyfield J, Petrukhin K. Bestrophin, the product of the Best vitelliform macular dystrophy gene (VMD2), localizes to the basolateral plasma membrane of the retinal pigment epithelium. Proc Natl Acad Sci U S A. 2000;97:12758-63 pubmed
Sripathi S, Hu M, Turaga R, Mertz J, Liu M, Wan J, et al. Proteome Landscape of Epithelial-to-Mesenchymal Transition (EMT) of Retinal Pigment Epithelium Shares Commonalities With Malignancy-Associated EMT. Mol Cell Proteomics. 2021;20:100131 pubmed publisher
Afanasyeva T, Corral Serrano J, Garanto A, Roepman R, Cheetham M, Collin R. A look into retinal organoids: methods, analytical techniques, and applications. Cell Mol Life Sci. 2021;78:6505-6532 pubmed publisher
Schustak J, Twarog M, Wu X, Wu H, Huang Q, Bao Y. Mechanism of Nucleic Acid Sensing in Retinal Pigment Epithelium (RPE): RIG-I Mediates Type I Interferon Response in Human RPE. J Immunol Res. 2021;2021:9975628 pubmed publisher
Choudhury R, Bayatti N, Scharff R, Szula E, Tilakaratna V, Udsen M, et al. FHL-1 interacts with human RPE cells through the α5β1 integrin and confers protection against oxidative stress. Sci Rep. 2021;11:14175 pubmed publisher
Zhao Q, Kong Y, Kittredge A, Li Y, Shen Y, Zhang Y, et al. Distinct expression requirements and rescue strategies for BEST1 loss- and gain-of-function mutations. elife. 2021;10: pubmed publisher
Tsai Y, Li Y, Ryu J, Su P, Cheng C, Wu W, et al. Impaired cholesterol efflux in retinal pigment epithelium of individuals with juvenile macular degeneration. Am J Hum Genet. 2021;108:903-918 pubmed publisher
D Antonio Chronowska A, D Antonio M, Frazer K. In vitro Differentiation of Human iPSC-derived Retinal Pigment Epithelium Cells (iPSC-RPE). Bio Protoc. 2019;9:e3469 pubmed publisher
Biswas P, Borooah S, Matsui H, Voronchikhina M, Zhou J, Zawaydeh Q, et al. Detection and validation of novel mutations in MERTK in a simplex case of retinal degeneration using WGS and hiPSC-RPEs model. Hum Mutat. 2021;42:189-199 pubmed publisher
Bonilha V, Bell B, DeBenedictis M, Hagstrom S, Fishman G, Hollyfield J. Cellular Changes in Retinas From Patients With BEST1 Mutations. Front Cell Dev Biol. 2020;8:573330 pubmed publisher
Schäfer N, Wolf H, Enzbrenner A, Schikora J, Reichenthaler M, Enzmann V, et al. Properdin Modulates Complement Component Production in Stressed Human Primary Retinal Pigment Epithelium Cells. Antioxidants (Basel). 2020;9: pubmed publisher
Mitrousis N, Hacibekiroglu S, Ho M, Sauve Y, Nagy A, van der Kooy D, et al. Hydrogel-mediated co-transplantation of retinal pigmented epithelium and photoreceptors restores vision in an animal model of advanced retinal degeneration. Biomaterials. 2020;257:120233 pubmed publisher
Zhang Z, Yan B, Gao F, Li Q, Meng X, Chen P, et al. PSCs Reveal PUFA-Provoked Mitochondrial Stress as a Central Node Potentiating RPE Degeneration in Bietti's Crystalline Dystrophy. Mol Ther. 2020;28:2642-2661 pubmed publisher
Udry F, Decembrini S, Gamm D, D xe9 glon N, Kostic C, Arsenijevic Y. Lentiviral mediated RPE65 gene transfer in healthy hiPSCs-derived retinal pigment epithelial cells markedly increased RPE65 mRNA, but modestly protein level. Sci Rep. 2020;10:8890 pubmed publisher
Mladenov N, Petrova S, Mladenova K, Bozhinova D, Moskova Doumanova V, Topouzova Hristova T, et al. Miscibility of hBest1 and sphingomyelin in surface films - A prerequisite for interaction with membrane domains. Colloids Surf B Biointerfaces. 2020;189:110893 pubmed publisher
Voisin A, Monville C, Plancheron A, Béré E, Gaillard A, Leveziel N. Cathepsin B pH-Dependent Activity Is Involved in Lysosomal Dysregulation in Atrophic Age-Related Macular Degeneration. Oxid Med Cell Longev. 2019;2019:5637075 pubmed publisher
Ji C, Li Y, Kittredge A, Hopiavuori A, Ward N, Yao P, et al. Investigation and Restoration of BEST1 Activity in Patient-derived RPEs with Dominant Mutations. Sci Rep. 2019;9:19026 pubmed publisher
Smith E, D Antonio Chronowska A, Greenwald W, Borja V, Aguiar L, Pogue R, et al. Human iPSC-Derived Retinal Pigment Epithelium: A Model System for Prioritizing and Functionally Characterizing Causal Variants at AMD Risk Loci. Stem Cell Reports. 2019;12:1342-1353 pubmed publisher
Shahi P, Hermans D, Sinha D, Brar S, Moulton H, Stulo S, et al. Gene Augmentation and Readthrough Rescue Channelopathy in an iPSC-RPE Model of Congenital Blindness. Am J Hum Genet. 2019;104:310-318 pubmed publisher
Zhang Y, Kittredge A, Ward N, Ji C, Chen S, Yang T. ATP activates bestrophin ion channels through direct interaction. Nat Commun. 2018;9:3126 pubmed publisher
Voisin A, Monville C, Plancheron A, Balbous A, Gaillard A, Leveziel N. hRPE cells derived from induced pluripotent stem cells are more sensitive to oxidative stress than ARPE-19 cells. Exp Eye Res. 2018;177:76-86 pubmed publisher
Ramachandra Rao S, Pfeffer B, Más Gómez N, Skelton L, Keiko U, Sparrow J, et al. Compromised phagosome maturation underlies RPE pathology in cell culture and whole animal models of Smith-Lemli-Opitz Syndrome. Autophagy. 2018;14:1796-1817 pubmed publisher
Li Y, Zhang Y, Xu Y, Kittredge A, Ward N, Chen S, et al. Patient-specific mutations impair BESTROPHIN1's essential role in mediating Ca2+-dependent Cl- currents in human RPE. elife. 2017;6: pubmed publisher
Zhang Y, Zhang D, Wei W, Shen B, Wang Y, Zhang Y, et al. Effects of RPE-conditioned medium on the differentiation of hADSCs into RPE cells, and their proliferation and migration. Exp Ther Med. 2017;14:3699-3707 pubmed publisher
Klingeborn M, Dismuke W, Skiba N, Kelly U, Stamer W, Bowes Rickman C. Directional Exosome Proteomes Reflect Polarity-Specific Functions in Retinal Pigmented Epithelium Monolayers. Sci Rep. 2017;7:4901 pubmed publisher
Shim S, Kim G, Lee D, Lee J, Kwon H, Song W. Survival of Transplanted Human Embryonic Stem Cell-Derived Retinal Pigment Epithelial Cells in a Human Recipient for 22 Months. JAMA Ophthalmol. 2017;135:287-289 pubmed publisher
Kaneko Y, Pappas C, Tajiri N, Borlongan C. Oxytocin modulates GABAAR subunits to confer neuroprotection in stroke in vitro. Sci Rep. 2016;6:35659 pubmed publisher
Parker J, Mitrousis N, Shoichet M. Hydrogel for Simultaneous Tunable Growth Factor Delivery and Enhanced Viability of Encapsulated Cells in Vitro. Biomacromolecules. 2016;17:476-84 pubmed publisher
Doumanov J, Zeitz C, Domínguez Giménez P, Audo I, Krishna A, Alfano G, et al. Disease-causing mutations in BEST1 gene are associated with altered sorting of bestrophin-1 protein. Int J Mol Sci. 2013;14:15121-40 pubmed publisher
Maruotti J, Wahlin K, Gorrell D, Bhutto I, Lutty G, Zack D. A simple and scalable process for the differentiation of retinal pigment epithelium from human pluripotent stem cells. Stem Cells Transl Med. 2013;2:341-54 pubmed publisher
Blenkinsop T, Salero E, Stern J, Temple S. The culture and maintenance of functional retinal pigment epithelial monolayers from adult human eye. Methods Mol Biol. 2013;945:45-65 pubmed publisher
Davidson A, Millar I, Burgess Mullan R, Maher G, Urquhart J, Brown P, et al. Functional characterization of bestrophin-1 missense mutations associated with autosomal recessive bestrophinopathy. Invest Ophthalmol Vis Sci. 2011;52:3730-6 pubmed publisher
Davidson A, Millar I, Urquhart J, Burgess Mullan R, Shweikh Y, Parry N, et al. Missense mutations in a retinal pigment epithelium protein, bestrophin-1, cause retinitis pigmentosa. Am J Hum Genet. 2009;85:581-92 pubmed publisher
Mullins R, Oh K, Heffron E, Hageman G, Stone E. Late development of vitelliform lesions and flecks in a patient with best disease: clinicopathologic correlation. Arch Ophthalmol. 2005;123:1588-94 pubmed
product information
master code :
NB300-164
SKU :
NB300-164
product name :
Bestrophin 1 Antibody (E6-6) - BSA Free
unit size :
0.1 ml (also 0.025 ml)
description :
The Bestrophin 1 Antibody (E6-6) - BSA Free from Novus is a mouse monoclonal antibody to Bestrophin 1. This antibody reacts with canine,human,mouse,porcine,primate,rat,rat (negative). The Bestrophin 1 Antibody (E6-6) - BSA Free has been validated for the following applications: Immunoprecipitation,Western Blot,Immunohistochemistry-Frozen,Immunocytochemistry/ Immunofluorescence,IF/ICC,IF/IHC,Immunohistochemistry-Paraffin,Immunohistochemistry,Proximity Ligation Assay,Knockout Validated,Dual RNAscope ISH-IHC.
target :
Bestrophin 1
category :
Primary Antibodies
buffer :
PBS
clonality :
Monoclonal
clone :
E6-6
concentration :
1.0 mg/ml
conjugate :
Unconjugated
host :
Mouse
immunogen :
Synthetic peptide conjugated to KLH corresponding to the C-terminus of human Bestrophin 1 (KDHMDPYWALENRDEAHS) [Uniprot: O76090]
isotype :
IgG1 Kappa
purity :
Protein A or G purified
species :
Canine,Human,Mouse,Porcine,Primate,Rat,Rat (Negative)
gene symbol :
BEST1
Antibody validation :
Knockout/Knockdown
accessionNumbers :
O76090
applications :
IF/ICC,IF/IHC,Immunoprecipitation,Western Blot,Immunohistochemistry-Paraffin,Immunohistochemistry-Frozen,Immunocytochemistry/ Immunofluorescence,Immunohistochemistry,Proximity Ligation Assay,Knockout Validated,Dual RNAscope ISH-IHC
USD :
479 USD
alt names :
ARB, BEST, Best disease, bestrophin 1, bestrophin-1, BMD, TU15B, vitelliform macular dystrophy 2, Vitelliform macular dystrophy protein 2, VMD2RP50
storage :
Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.
more info or order :
company information
Novus Biologicals
10771 E Easter Ave
Centennial, CO 80112
novus@novusbio.com
https://www.novusbio.com
3037301950
headquarters: USA
Novus Biologicals licenses, manufactures, and markets antibodies to over 20,000 unique targets to support a wide array of research areas. Novus is built on honesty, collaboration and strong relationships and continues to provide quality tools that accelerate research. Every product is backed by our 100% guarantee.