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Physiology 20: 292-302, 2005; doi:10.1152/physiol.00021.2005
1548-9213/05 $8.00
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Physiology, Vol. 20, No. 5, 292-302, October 2005
© 2005 Int. Union Physiol. Sci./Am. Physiol. Soc.

EMERGING TOPICS

Looking Chloride Channels Straight in the Eye: Bestrophins, Lipofuscinosis, and Retinal Degeneration

Criss Hartzell, Zhiqiang Qu, Ilva Putzier, Liana Artinian, Li-Ting Chien and Yuanyuan Cui

Department of Cell Biology, The Center for Neurodegenerative Disease, Emory University School of Medicine, Atlanta, Georgia

criss.hartzell{at}emory.edu

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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