Source: | Rabbit | Gene Id: | 5068 |
Isotype: | IgG | Swiss Prot: | |
purity: | Purified by Protein A. |
Background: |
Best vitelliform macular dystrophy, known as Best disease, is an early-onset autosomal dominant condition in which accumulation of lipofuscin-like material within and beneath the RPE leads to progressive loss of central vision. Best disease is frequently a reflection of mutations in the Bestrophin gene, which encodes a protein containing four putative transmembrane domains and localizes to the basolateral plasma membrane of RPE cells. Human Bestrophin forms oligomeric chloride channels that are sensitive to intracellular calcium. Missense mutations at the Bestrophin locus reduces or abolishes Bestrophin protein mediated membrane current. Bestrophin Bestrophin 2,Bestrophin 3, and Bestrophin 4 are transmembrane proteins that contain a high percentage of aromatic residues, a conserved RFP (Arg-Phe-Pro) motif and they function as anion channels. |
Reactivity | Human, Mouse, Rat |
Tested applications | WB IHC IF |
Clonality | Polyclonal Antibody |
Calculated MW | / |
Recommended Dilutions |
WB 1:100-1:1000
IHC 1:100-1:500
IF 1:50-1:200
|
Immunogen | KLH conjugated synthetic peptide derived from human Bestrophin |
Storage | Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months |
Synonym | BEST 1, BEST1, BEST-1, BEST, Best macular dystrophy, BEST1, BEST1_HUMAN, Bestrophin 1, Bestrophin-1, Bestrophin1, BMD, mBest1, TU15B, Vitellorm macular dystrophy 2, Vitellorm macular dystrophy, Vitellorm macular dystrophy protein 2, VMD 2, VMD2. |
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