1. CLC Chloride Channels: Structure, Function, and Dysfunction THOMAS J. JENTSCH, Zentrum für Molekulare Neurobiologie, Universität Hamburg, Falkenried 94, D-20246 Hamburg, Germany (Sponsor: Criss Hartzell)
The CLC gene family, originally identified by expression cloning of ClC-0 from Torpedo, is the only known gene family of voltage-gated Cl− channels and has members in all phylae. The crystal structure of bacterial CLC proteins, which was recently elucidated by Dutzler et al., confirmed the double-barreled structure that was previously supported by single-channel recordings and mutagenesis of ClC-0. It provided fascinating insights into the anion-dependent gating mechanism. The bacterial structure was used to guide us in the recent characterization of an inhibitor binding site in ClC-1. Only recently binding partners were identified, e.g., barttin, a β-subunit of ClC-K channels that is necessary for surface expression.
CLC channels have broad physiological functions, as evident from human disease and mouse models. ClC-1 is...
