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Bestrophin 1 Products

(Bestrophin 1 (BEST1))

Categories

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.[provided by RefSeq, Nov 2008].

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Featured Bestrophin 1 Categories

Bestrophin 1 Antibodies

High quality antibodies with extensive validation data.

Bestrophin 1 Proteins

Proteins for various applications incl. WB, ELISA, IF etc.

Recommended Bestrophin 1 Antibodies

Product
Reactivity
Application
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human, Mouse, Rat
Application IHC, WB
Validations
  • (4)
Cat. No. ABIN7260777
Quantity 200 μL
Datasheet Datasheet
Reactivity Human
Application ELISA, IHC, WB
Validations
  • (2)
Cat. No. ABIN7189978
Quantity 100 μL
Datasheet Datasheet
Reactivity Human
Application ICC, IHC, WB
Validations
  • (2)
Cat. No. ABIN5013231
Quantity 100 μL
Datasheet Datasheet

Recommended Bestrophin 1 ELISA Kits

Product
Reactivity
Analytical Method
Validations
Cat. No.
Quantity
Datasheet
Reactivity Human
Analytical Method Quantitative Sandwich ELISA
Validations
Cat. No. ABIN6202076
Quantity 96 tests
Datasheet Datasheet

Recommended Bestrophin 1 Proteins

Product
Reactivity
Source
Validations
Cat. No.
Quantity
Datasheet
Reactivity Mouse
Source Insect Cells
Validations
  • (1)
Cat. No. ABIN3132248
Quantity 1 mg
Datasheet Datasheet
Reactivity Human
Source Insect Cells
Validations
  • (1)
Cat. No. ABIN3089793
Quantity 1 mg
Datasheet Datasheet
Reactivity Mouse
Source Insect Cells
Validations
  • (1)
Cat. No. ABIN3132247
Quantity 1 mg
Datasheet Datasheet

Latest Publications for our Bestrophin 1 products

Voisin, Monville, Plancheron, Balbous, Gaillard, Leveziel: "hRPE cells derived from induced pluripotent stem cells are more sensitive to oxidative stress than ARPE-19 cells." in: Experimental eye research, Vol. 177, pp. 76-86, (2019) (PubMed).

Shahi, Hermans, Sinha, Brar, Moulton, Stulo, Borys, Capowski, Pillers, Gamm, Pattnaik: "Gene Augmentation and Readthrough Rescue Channelopathy in an iPSC-RPE Model of Congenital Blindness." in: American journal of human genetics, Vol. 104, Issue 2, pp. 310-318, (2019) (PubMed).

Zhang, Kittredge, Ward, Ji, Chen, Yang: "ATP activates bestrophin ion channels through direct interaction." in: Nature communications, Vol. 9, Issue 1, pp. 3126, (2018) (PubMed).

Shim, Kim, Lee, Lee, Kwon, Song: "Survival of Transplanted Human Embryonic Stem Cell-Derived Retinal Pigment Epithelial Cells in a Human Recipient for 22 Months." in: JAMA ophthalmology, Vol. 135, Issue 3, pp. 287-290, (2017) (PubMed).

Uggenti, Briant, Streit, Thomson, Koay, Baines, Swanton, Manson: "Restoration of mutant bestrophin-1 expression, localisation and function in a polarised epithelial cell model." in: Disease models & mechanisms, Vol. 9, Issue 11, pp. 1317-1328, (2016) (PubMed).

Parker, Mitrousis, Shoichet: "Hydrogel for Simultaneous Tunable Growth Factor Delivery and Enhanced Viability of Encapsulated Cells in Vitro." in: Biomacromolecules, Vol. 17, Issue 2, pp. 476-84, (2016) (PubMed).

Kaneko, Pappas, Tajiri, Borlongan: "Oxytocin modulates GABAAR subunits to confer neuroprotection in stroke in vitro." in: Scientific reports, Vol. 6, pp. 35659, (2016) (PubMed).

Kuo, Abdullaev, Hyzinski-García, Mongin: "Effects of alternative splicing on the function of bestrophin-1 calcium-activated chloride channels." in: The Biochemical journal, Vol. 458, Issue 3, pp. 575-83, (2014) (PubMed).

Sharon, Al-Hamdani, Engelsberg, Mizrahi-Meissonnier, Obolensky, Banin, Sander, Jensen, Larsen, Schatz: "Ocular phenotype analysis of a family with biallelic mutations in the BEST1 gene." in: American journal of ophthalmology, Vol. 157, Issue 3, pp. 697-709.e1-2, (2014) (PubMed).

Bertolotti, Neri, Camparini, Macaluso, Marigo: "Stem cells as source for retinal pigment epithelium transplantation." in: Progress in retinal and eye research, Vol. 42, pp. 130-44, (2014) (PubMed).

Synonyms and alternative names related to Bestrophin 1

Bestrophin 1 (Best1), bestrophin 1 (BEST1), bestrophin 1 (best1), Bestrophin 1 (Bm1_25500), bestrophin 1 (Best1), anon-WO0118547.380, ARB, best, BEST, best-1, BEST1, BMD, Bmd, CG6264, Dbest, dBest1, dbest1, dmBest1, Dmel\\CG6264, mBest1, RP50, TU15B, VMD2, Vmd2

Protein level used designations for Bestrophin 1

  • Best1-PA
  • Best1-PB
  • Best1-PC
  • Best1-PD
  • CG6264-PA
  • CG6264-PB
  • CG6264-PC
  • CG6264-PD
  • bestrophin 1
  • bestrophin-1
  • Bestrophin 1
  • bestrophin-1-like
  • Best disease
  • Best1V1Delta2
  • vitelliform macular dystrophy protein 2
  • best macular dystrophy
  • vitelliform macular dystrophy 2 homolog
  • vitelliform macular dystrophy protein 2 homolog
  • vitelliform macular dystrophy (Best disease, bestrophin)
  • Bestrophin-1
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