Molecular weight (DA)
67kD
Immunogen
Synthetic Peptide of Bestrophin-1 AA range: 161-211
Specificity
The antibody detects endogenous Bestrophin-1 protein
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB: 1/500 - 1/2000. IHC: 1/100 - 1/300. ELISA: 1/40000.. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
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],
Function
disease:Defects in BEST1 are a cause of adult-onset vitelliform macular dystrophy (AVMD) [MIM:608161]. AVMD is a rare autosomal dominant disorder with incomplete penetrance and highly variable expression. Patients usually become symptomatic in the fourth or fifth decade of life with a protracted disease of decreased visual acuity.,disease:Defects in BEST1 are the cause of autosomal recessive bestrophinopathy (ARB) [MIM:611809]. ARB is associated with central visual loss, a characteristic retinopathy, an absent electro-oculogram light rise, and a reduced electroretinogram.,disease:Defects in BEST1 are the cause of vitelliform macular dystrophy type 2 (VMD2) [MIM:153700]; also known as Best macular dystrophy (BMD). VMD2 is an autosomal dominant form of macular degeneration that usually begins in childhood or adolescence. VMD2 is characterized by typical "egg-yolk" macular lesions due to ab
Protein name
Bestrophin-1 (TU15B) (Vitelliform macular dystrophy protein 2)
Abbreviation
Bestrophin-1
Other name
Bestrophin-1 (TU15B;Vitelliform macular dystrophy protein 2)
Human protein sequence Database
O76090
Mouse protein sequence database
O88870
Rat protein sequence database
Cellular localization
Cell membrane ; Multi-pass membrane protein . Basolateral cell membrane .
Tissue expression
Predominantly expressed in the basolateral membrane of the retinal pigment epithelium.
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