Recent evidence suggests that Cl ion channels are important for retinal integrity. Bestrophin Cl channel mutations in humans are genetically linked to a juvenile form of macular degeneration, and disruption of some ClC Cl channels in mice leads to retinal degeneration. In both cases, accumulation of lipofuscin pigment is a key feature of the cellular degeneration. Because Cl channels regulate the ionic environment inside organelles in the endosomal-lysosomal pathway, retinal degeneration may result from defects in lysosomal trafficking or function.

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