Structure and selectivity in bestrophin ion channels

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Science  17 Oct 2014:
Vol. 346, Issue 6207, pp. 355-359
DOI: 10.1126/science.1259723

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Insight into a retinal degeneration disease

Human bestrophin 1 (hBest1) is a membrane protein that forms a chloride channel in the retinal pigment epithelium. Mutations in hBest1 can lead to a retinal degeneration disease known as Best disease. Yang et al. describe the structure of KpBest, a bacterial homolog of hBest1. KpBest forms a pentamer with an ion channel at its center. In contrast to hBest1, KpBest1 is a sodium channel. The structure suggests a mechanism for ion selectivity that was confirmed by mutagenesis of KpBest and hBest1. A model of the hBest1 channel structure based on the KpBest structure reveals how mutations cause disease.

Science, this issue p. 355