Criss Hartzell , Zhiqiang Qu , Ilva Putzier , Liana Artinian , Li - Ting Chien and Yuanyuan Cui Lipofuscinosis , and Retinal Degeneration Looking Chloride Channels Straight
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منابع مشابه
The anion-selective pore of the bestrophins, a family of chloride channels associated with retinal degeneration.
Mutations in human bestrophin-1 (VMD2) are genetically linked to a juvenile form of macular degeneration and autosomal dominant vitreoretinochoroidopathy. Recently, it has been proposed that bestrophins are Cl- channels and that the putative second transmembrane domain participates in forming the bestrophin pore. However, the structural determinants of Cl- ion permeation through the channel por...
متن کاملMacular Degeneration is Often Associated with Retinal Lipofuscinosis
neous mixture of both proteins and lipids that are partially oxidized (67). It is thought that lipofuscin accumulation is a major causal factor in AMD because it correlates with the severity of photoreceptor degeneration (94) and has been shown to have toxic properties (21, 68). The major fluorescent component of lipofuscin is Nretinylidene-N-retinylethanolamine (A2E), which is a metabolite of ...
متن کاملCalcium-activated chloride channels.
Calcium-activated chloride channels (CaCCs) play important roles in cellular physiology, including epithelial secretion of electrolytes and water, sensory transduction, regulation of neuronal and cardiac excitability, and regulation of vascular tone. This review discusses the physiological roles of these channels, their mechanisms of regulation and activation, and the mechanisms of anion select...
متن کاملMolecular physiology of bestrophins: multifunctional membrane proteins linked to best disease and other retinopathies.
This article reviews the current state of knowledge about the bestrophins, a newly identified family of proteins that can function both as Cl(-) channels and as regulators of voltage-gated Ca(2+) channels. The founding member, human bestrophin-1 (hBest1), was identified as the gene responsible for a dominantly inherited, juvenile-onset form of macular degeneration called Best vitelliform macula...
متن کاملCellular Biology Explaining Calcium-Dependent Gating of Anoctamin-1 Chloride Channels Requires a Revised Topology
channels were recently discovered to be encoded by members of the anoctamin (Ano, also called Tmem16) superfamily, but the mechanisms of Ano1 gating by Ca remain enigmatic. Objective: The objective was to identify regions of Ano1 involved in channel gating by Ca . Methods and Results: The Ca sensitivity of Ano1 was estimated from rates of current activation, and deactivation in excised patches ...
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تاریخ انتشار 2005