Bestrophin 1 Produits
(Bestrophin 1 (BEST1))
Catégories
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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Catégories Bestrophin 1 en vedette
Bestrophin 1 Anticorps
High quality antibodies with extensive validation data.
Bestrophin 1 Protéines
Proteins for various applications incl. WB, ELISA, IF etc.
Bestrophin 1 recommandé Anticorps
Produit
Reactivity
Application
Validations
N° du produit
Quantité
Fiche technique
Reactivity
Human, Mouse, Rat
Application
WB, IHC
Validations
- (4)
N° du produit
ABIN7260777
Quantité
200 μL
Fiche technique
Fiche technique
Reactivity
Human
Application
WB
Validations
- (4)
N° du produit
ABIN1678855
Quantité
100 μg
Fiche technique
Fiche technique
Reactivity
Human
Application
WB, IHC, ELISA
Validations
- (2)
N° du produit
ABIN7189978
Quantité
100 μL
Fiche technique
Fiche technique
Bestrophin 1 recommandé Kits ELISA
Produit
Reactivity
Analytical Method
Validations
N° du produit
Quantité
Fiche technique
Reactivity
Human
Analytical Method
Quantitative Sandwich ELISA
Validations
N° du produit
ABIN6202076
Quantité
96 tests
Fiche technique
Fiche technique
Bestrophin 1 recommandé Protéines
Produit
Reactivity
Source
Validations
N° du produit
Quantité
Fiche technique
Reactivity
Human
Source
Wheat germ
Validations
- (1)
N° du produit
ABIN1346633
Quantité
10 μg
Fiche technique
Fiche technique
Reactivity
Human
Source
Wheat germ
Validations
- (1)
N° du produit
ABIN1346631
Quantité
10 μg
Fiche technique
Fiche technique
Reactivity
Human
Source
Escherichia coli (E. coli)
Validations
N° du produit
ABIN7419085
Quantité
100 μg
Fiche technique
Fiche technique
Dernières publications sur nos produits Bestrophin 1
: "hRPE cells derived from induced pluripotent stem cells are more sensitive to oxidative stress than ARPE-19 cells." dans: Experimental eye research, Vol. 177, pp. 76-86, (2019) (PubMed).: "Gene Augmentation and Readthrough Rescue Channelopathy in an iPSC-RPE Model of Congenital Blindness." dans: American journal of human genetics, Vol. 104, Issue 2, pp. 310-318, (2019) (PubMed).
: "ATP activates bestrophin ion channels through direct interaction." dans: Nature communications, Vol. 9, Issue 1, pp. 3126, (2018) (PubMed).
: "Survival of Transplanted Human Embryonic Stem Cell-Derived Retinal Pigment Epithelial Cells in a Human Recipient for 22 Months." dans: JAMA ophthalmology, Vol. 135, Issue 3, pp. 287-290, (2017) (PubMed).
: "Restoration of mutant bestrophin-1 expression, localisation and function in a polarised epithelial cell model." dans: Disease models & mechanisms, Vol. 9, Issue 11, pp. 1317-1328, (2016) (PubMed).
: "Hydrogel for Simultaneous Tunable Growth Factor Delivery and Enhanced Viability of Encapsulated Cells in Vitro." dans: Biomacromolecules, Vol. 17, Issue 2, pp. 476-84, (2016) (PubMed).
: "Oxytocin modulates GABAAR subunits to confer neuroprotection in stroke in vitro." dans: Scientific reports, Vol. 6, pp. 35659, (2016) (PubMed).
: "Effects of alternative splicing on the function of bestrophin-1 calcium-activated chloride channels." dans: The Biochemical journal, Vol. 458, Issue 3, pp. 575-83, (2014) (PubMed).
: "Ocular phenotype analysis of a family with biallelic mutations in the BEST1 gene." dans: American journal of ophthalmology, Vol. 157, Issue 3, pp. 697-709.e1-2, (2014) (PubMed).
: "Stem cells as source for retinal pigment epithelium transplantation." dans: Progress in retinal and eye research, Vol. 42, pp. 130-44, (2014) (PubMed).
Synonymes et noms alternatifs relatifs à 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, Vmd2Protein 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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