BEST1 monoclonal antibody, clone E6-6
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More Files
- More Functions
Western Blot (Cell lysate)
Western blot analysis of BEST1 (68 kDa) from human RPE cell lysate using BEST1 monoclonal antibody, clone E6-6 (Cat # MAB2330) (1 : 1000).
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Specification
Product Description
Mouse monoclonal antibody raised against synthetic peptide of BEST1.
Immunogen
A synthetic peptide (conjugated with KLH) corresponding to human BEST1.
Sequence
KDHMDPYWALENRDEAHS
Host
Mouse
Theoretical MW (kDa)
68
Reactivity
Human, Pig, Primates
Specificity
This antibody recognizes a band at ~68 KDa on Western Blot.
Form
Liquid
Isotype
IgG1, kappa
Recommend Usage
Western Blot (1:1000)
The optimal working dilution should be determined by the end user.Storage Buffer
In buffer containing 0.09% sodium azide
Storage Instruction
Store at -20°C or -80°C.
Aliquot to avoid repeated freezing and thawing.Note
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Applications
Western Blot (Cell lysate)
Western blot analysis of BEST1 (68 kDa) from human RPE cell lysate using BEST1 monoclonal antibody, clone E6-6 (Cat # MAB2330) (1 : 1000).Immunofluorescence
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Gene Info — BEST1
Entrez GeneID
7439Protein Accession#
O76090Gene Name
BEST1
Gene Alias
ARB, BEST, BMD, TU15B, VMD2
Gene Description
bestrophin 1
Gene Ontology
HyperlinkGene Summary
This gene encodes 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
Other Designations
Best disease|vitelliform macular dystrophy protein 2
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Interactome
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Disease
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Publication Reference
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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 Jan; 6(3):217.
Application：IF, WB, Human, Human embryonic stem (hES) cell–derived putative retinal pigment epithelium (RPE).
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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.
PNAS 2000 Nov; 97(23):12758.
Application：ICC, IF, IHC-P, IP, WB-Ce, WB-Ti, Human, Monkey, Pig, Human ocular tissuess, Retinal pigment epitheliumm, RPE, RPE-J cells.
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Bestrophin gene mutations in patients with Best vitelliform macular dystrophy.
Caldwell GM, Kakuk LE, Griesinger IB, Simpson SA, Nowak NJ, Small KW, Maumenee IH, Rosenfeld PJ, Sieving PA, Shows TB, Ayyagari R.
Genomics 1999 May; 58(1):98.
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Identification of the gene responsible for Best macular dystrophy.
Petrukhin K, Koisti MJ, Bakall B, Li W, Xie G, Marknell T, Sandgren O, Forsman K, Holmgren G, Andreasson S, Vujic M, Bergen AA, McGarty-Dugan V, Figueroa D, Austin CP, Metzker ML, Caskey CT, Wadelius C.
Nature Genetics 1998 Jul; 19(3):241.
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Derivation and comparative assessment of retinal pigment epithelium from human embryonic stem cells using transcriptomics.
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