Referenced in: none

Automatically associated channels: ClC4

Title: Looking chloride channels straight in the eye: bestrophins, lipofuscinosis, and retinal degeneration.

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

Journal, date & volume: , 2005 Oct , 20, 292-302

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/16174869

Abstract
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.