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Description
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).Synonyms: BEST1, TU15B, VMD2, Vitelliform macular dystrophy protein 2
Immunogen
Synthetic peptide corresponding to amino acids 595-614 of Human Bestrophin. Epitope: C-terminal