Abstract
The bestrophin family of anion channels consists of four mammalian paralogs, namely bestrophin1-4 (Best1-4), at least two members of which play important functions in the eye. Best1 is predominantly expressed in the retinal pigment epithelium (RPE) and is required for generating a vision-related electrical signal named “light peak”. Mutations in the human BEST1 gene have been genetically linked to a spectrum of untreatable retinal degenerative disorders collectively known as bestrophinopathies. Meanwhile, Best2 is highly expressed in non-pigmented ciliary epithelial (NPE) cells, and Best2-/- mice exhibit a reduction of intra-ocular pressure (IOP), underlying the contribution of Best2 to IOP and the potential of Best2 as a drug target for relieving ocular hypertension.
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