Bestrophin 1

Bestrophin is a 68 kDa basolateral plasma membrane protein expressed in retinal pigment epithelial cells (RPE).   Bestrophin is encoded by the VMD2 gene, which is mutated in Best macular dystrophy, a disease characterized by a depressed light peak due to alteration in the chloride channel activity and changes in the electrooculogram (EOG).  Recently it was proposed that bestrophin is a chloride channel responsible for generating the light peak.  It has been shown that Bestrophin interacts with beta catalytic subunit of protein phosphatase 2A (PP2Ac).  Such Protein-protein interaction between bestrophin and PP2Ac and the structural subunit of PP2A, PR65, was confirmed by reciprocal immunoprecipitation.   The interaction between PP2Ac and the Bestrophin takes place near the Carboxy-terminal end of the protein.  Okadic acid induce the phosphorylation of Bestrophin in vitro.  Bestrophin also serves in the signal transduction pathway that modulates the light peak of the EOG, that is regulated by phosphorylation of the Bestrophin that in turn is regulated by protein phosphatase 2A (PP2A). 

The Anti-Bestrophin-selective antibodies were generated against conserved sequences near the N-terminal, mid-region and the C-terminal end of the proteins that are unique to Bestrophin protein.  The Bestrophin-selective antibodies were affinity purified against immobilized antigen based affinity chromatography and are represented as epitope-specific antibodies.  The polyclonal antibodies strongly labels a 65 kDa protein in RPE cell extracts.  Anti-Bestrophin-selective antibodies are also available in affinity-purified form for confocal, Western blotting and immunocytochemical analyses.  FabGennix Inc. will also conjugate antibodies with fluorescent probes upon request at extra charge.   FabGennix Inc. also provides antibodies against proteins that are involved in Best disease, Usher syndrome and macular dystrophy conditions.  Some of our products in this suite of antibodies include various Anti-PDE antibodies; Anti-MerTK, Anti-Phospho-MERTK, Anti-Usher; Anti-ELVLO4; Anti-Myocilin; Anti-Orphan Receptor GP75 and many more.  FabGennix Int. Inc.,  employs cyclic peptide methodology for generating high specificity and affinity antibodies (6).  FabGennix Int. Inc., will also provide Western blot positive controls for most of these antibodies in ready-to-use buffer for easy identification of respective proteins.  Limited quantities of antigens are also available.  Please enquire for their availability before ordering.

For research use only, not for diagnostic or therapeutic use.