Article
Molecular basis of ClC-6 function and its impairment in human disease.
Science advances - 13 Oct 2023
Zhang Bing, Zhang Sensen, Polovitskaya Maya M, Yi Jingbo, Ye Binglu, Li Ruochong, Huang Xueying, Yin Jian, Neuens Sebastian, Balfroid Tom, Soblet Julie, Vens Daphné, Aeby Alec, Li Xiaoling, Cai Jinjin, Song Yingcai, Li Yuanxi, Tartaglia Marco, Li Yang, Jentsch Thomas J, Yang Maojun, Liu Zhiqiang
Abstract excerpt
ClC-6 is a late endosomal voltage-gated chloride-proton exchanger that is predominantly expressed in the nervous system. Mutated forms of ClC-6 are associated with severe neurological disease. However, the mechanistic role of ClC-6 in normal and pathological states remains largely unknown. Here, we present cryo-EM structures of ClC-6 that guided subsequent functional studies. Previously unrecognized ATP binding...
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