NB300-164

Mladenov N, Petrova SD, Mladenova K, Bozhinova D, Moskova-Doumanova V, Topouzova-Hristova T, Videv P, Veleva R, Kostadinova A, Staneva G, Andreeva TD, Doumanov JA
Colloids and surfaces. B, Biointerfaces 2020 May;189:110893

Cathepsin B pH-Dependent Activity Is Involved in Lysosomal Dysregulation in Atrophic Age-Related Macular Degeneration.

Voisin A, Monville C, Plancheron A, Béré E, Gaillard A, Leveziel N
Oxidative medicine and cellular longevity 2019;2019:5637075

Gene Augmentation and Readthrough Rescue Channelopathy in an iPSC-RPE Model of Congenital Blindness.

Shahi PK, Hermans D, Sinha D, Brar S, Moulton H, Stulo S, Borys KD, Capowski E, Pillers DM, Gamm DM, Pattnaik BR
American journal of human genetics 2019 Feb 7;104(2):310-318

Investigation and Restoration of BEST1 Activity in Patient-derived RPEs with Dominant Mutations.

Ji C, Li Y, Kittredge A, Hopiavuori A, Ward N, Yao P, Fukuda Y, Zhang Y, Tsang SH, Yang T
Scientific reports 2019 Dec 13;9(1):19026

Compromised phagosome maturation underlies RPE pathology in cell culture and whole animal models of Smith-Lemli-Opitz Syndrome.

Ramachandra Rao S, Pfeffer BA, Más Gómez N, Skelton LA, Keiko U, Sparrow JR, Rowsam AM, Mitchell CH, Fliesler SJ
Autophagy 2018;14(10):1796-1817

hRPE cells derived from induced pluripotent stem cells are more sensitive to oxidative stress than ARPE-19 cells.

Voisin A, Monville C, Plancheron A, Balbous A, Gaillard A, Leveziel N
Experimental eye research 2018 Dec;177:76-86

ATP activates bestrophin ion channels through direct interaction.

Zhang Y, Kittredge A, Ward N, Ji C, Chen S, Yang T
Nature communications 2018 Aug 7;9(1):3126

Effects of RPE-conditioned medium on the differentiation of hADSCs into RPE cells, and their proliferation and migration.

Zhang Y, Zhang D, Wei W, Shen B, Wang Y, Zhang Y, Zhang Y, Ji J, Sun H, Luo M, Gu P
Experimental and therapeutic medicine 2017 Oct;14(4):3699-3707

Patient-specific mutations impair BESTROPHIN1's essential role in mediating Ca(2+)-dependent Cl(-) currents in human RPE.

Li Y, Zhang Y, Xu Y, Kittredge A, Ward N, Chen S, Tsang SH, Yang T
eLife 2017 Oct 24;6

Survival of Transplanted Human Embryonic Stem Cell-Derived Retinal Pigment Epithelial Cells in a Human Recipient for 22 Months.

Shim SH, Kim G, Lee DR, Lee JE, Kwon HJ, Song WK
JAMA ophthalmology 2017 Mar 1;135(3):287-289

Directional Exosome Proteomes Reflect Polarity-Specific Functions in Retinal Pigmented Epithelium Monolayers.

Klingeborn M, Dismuke WM, Skiba NP, Kelly U, Stamer WD, Bowes Rickman C
Scientific reports 2017 Jul 7;7(1):4901

Oxytocin modulates GABA(A)R subunits to confer neuroprotection in stroke in vitro.

Kaneko Y, Pappas C, Tajiri N, Borlongan CV
Scientific reports 2016 Oct 21;6:35659

Restoration of mutant bestrophin-1 expression, localisation and function in a polarised epithelial cell model.

Uggenti C, Briant K, Streit AK, Thomson S, Koay YH, Baines RA, Swanton E, Manson FD
Disease models & mechanisms 2016 Nov 1;9(11):1317-1328

Hydrogel for Simultaneous Tunable Growth Factor Delivery and Enhanced Viability of Encapsulated Cells in Vitro.

Parker J, Mitrousis N, Shoichet MS
Biomacromolecules 2016 Feb 8;17(2):476-84

Stem cells as source for retinal pigment epithelium transplantation.

Bertolotti E, Neri A, Camparini M, Macaluso C, Marigo V
Progress in retinal and eye research 2014 Sep;42:130-44

Ocular phenotype analysis of a family with biallelic mutations in the BEST1 gene.

Sharon D, Al-Hamdani S, Engelsberg K, Mizrahi-Meissonnier L, Obolensky A, Banin E, Sander B, Jensen H, Larsen M, Schatz P
American journal of ophthalmology 2014 Mar;157(3):697-709.e1-2

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

A simple and scalable process for the differentiation of retinal pigment epithelium from human pluripotent stem cells.

Maruotti J, Wahlin K, Gorrell D, Bhutto I, Lutty G, Zack DJ
Stem cells translational medicine 2013 May;2(5):341-54

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 Sep;9(2):101-9

Generation of retinal pigment epithelial cells from small molecules and OCT4 reprogrammed human induced pluripotent stem cells.

Krohne TU, Westenskow PD, Kurihara T, Friedlander DF, Lehmann M, Dorsey AL, Li W, Zhu S, Schultz A, Wang J, Siuzdak G, Ding S, Friedlander M
Stem cells translational medicine 2012 Feb;1(2):96-109

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 Jun 23;52(7):4497-505

Functional characterization of bestrophin-1 missense mutations associated with autosomal recessive bestrophinopathy.

Davidson AE, Millar ID, Burgess-Mullan R, Maher GJ, Urquhart JE, Brown PD, Black GC, Manson FD
Investigative ophthalmology & visual science 2011 Jun 1;52(6):3730-6

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 Jan 7;6(1):e14499

Bestrophin 2 is expressed in human non-pigmented ciliary epithelium but not retinal pigment epithelium.

Zhang Y, Patil RV, Marmorstein AD
Molecular vision 2010 Feb 10;16:200-6

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 Apr;28(4):704-12

Long-term safety and function of RPE from human embryonic stem cells in preclinical models of macular degeneration.

Lu B, Malcuit C, Wang S, Girman S, Francis P, Lemieux L, Lanza R, Lund R
Stem cells (Dayton, Ohio) 2009 Sep;27(9):2126-35

Derivation of functional retinal pigmented epithelium from induced pluripotent stem cells.

Buchholz DE, Hikita ST, Rowland TJ, Friedrich AM, Hinman CR, Johnson LV, Clegg DO
Stem cells (Dayton, Ohio) 2009 Oct;27(10):2427-34

Missense mutations in a retinal pigment epithelium protein, bestrophin-1, cause retinitis pigmentosa.

Davidson AE, Millar ID, Urquhart JE, Burgess-Mullan R, Shweikh Y, Parry N, O'Sullivan J, Maher GJ, McKibbin M, Downes SM, Lotery AJ, Jacobson SG, Brown PD, Black GC, Manson FD
American journal of human genetics 2009 Nov;85(5):581-92

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 May;48(5):1959-67

Late development of vitelliform lesions and flecks in a patient with best disease: clinicopathologic correlation.

Mullins RF, Oh KT, Heffron E, Hageman GS, Stone EM
Archives of ophthalmology (Chicago, Ill. : 1960) 2005 Nov;123(11):1588-94

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(3):217-45

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 Aug 23;277(34):30591-7

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 Nov 7;97(23):12758-63

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Supportive validation

Submitted by: Novus Biologicals (provider)
Main image
Experimental details: Western Blot: Bestrophin 1 Antibody (E6-6) [NB300-164] - Western blot analysis of the normal and mutant human Best1 protein in transiently transfected MDCK cells. Best1 proteins are detectable as a 68 kDa band in all transfected cells, but not in non-transfected controls (MDCK lane). Actin bands are shown to indicate equal loading of cell lysates. Image collected and cropped by CiteAb from the following publication (http://www.mdpi.com/1422-0067/14/7/15121), licensed under a CC-BY licence.

Supportive validation

Submitted by: Novus Biologicals (provider)
Main image
Experimental details: Western Blot: Bestrophin 1 Antibody (E6-6) [NB300-164] - Detection of Bestrophin (68 kDa) from human RPE cell lysate.

Supportive validation

Submitted by: Novus Biologicals (provider)
Main image
Experimental details: Immunohistochemistry-Paraffin: Bestrophin 1 Antibody (E6-6) [NB300-164] - Bestrophin 1 was detected in immersion fixed paraffin-embedded sections of human brain using Mouse Anti-Human Bestrophin 1 (E6-6) Monoclonal Antibody (Catalog # NB300-164) at 1:300 for 1 hour at room temperature followed by incubation with the Anti-Mouse IgG VisUCyte™ HRP Polymer Antibody (Catalog # VC001). Tissue was stained using DAB (brown) and counterstained with hematoxylin (blue). Specific staining was localized to the cytoplasm in neurons.

Supportive validation

Submitted by: Novus Biologicals (provider)
Main image
Experimental details: Immunohistochemistry-Paraffin: Bestrophin 1 Antibody (E6-6) [NB300-164] - Bestrophin 1 was detected in immersion fixed paraffin sections of human small intestine using t Mouse Anti-Human Bestrophin 1 Monoclonal Antibody (Catalog # NB300-164) at 5 ug/mL for 1 hour at room temperature followed by incubation with the Anti-Mouse IgG VisUCyte™ HRP Polymer Antibody (Catalog # VC001). Tissue was stained using DAB (brown) and counterstained with hematoxylin (blue). Specific staining was localized to the cell surface and extracellular.