ISSN: 2041-6792

Clinical Investigation

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 This article reviews the present state of data about the bestrophins, a newly identified family of proteins which will function both as Cl− channels and as regulators of voltage-gated Ca2+ channels. The founding member, human bestrophin-1 (hBest1), was identified because the gene liable for a dominantly inherited, juvenile-onset sort of degeneration called Best vitelliform macular dystrophy. Mutations in hBest1 have also been related to a little fraction of adult-onset macular dystrophies.  In addition to functioning as a Cl− channel, hBest1 also is able to regulate voltage-gated Ca2+, but whether bestrophins are the molecular counterpart of Ca2+-activated Cl− channels remains unsure . Bestrophins are also regulated by cell volume and may be a member of the volume-regulated anion channel family.Cl− channels are ubiquitously expressed in both eukaryotic and prokaryotic cells. In eukaryotes, Cl− channels function both at the plasma membrane and in intracellular organelles. Physiologically, Cl− channels can be broadly classified as ligand gated, volume regulated (VRAC), voltage sensitive, or second messenger activated (e.g., cAMP or Ca2+). The molecular counterparts of some of these physiologically described channels are known. Ligand-gated anion channels include the GABAA and glycine receptors, voltage-gated anion channels include some members of the ClC family, and second messenger-activated channels include the cystic fibrosis transmembrane conductance regulator (CFTR).   

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