CLC chloride channels and transporters: structure, function, physiology, and disease

TJ Jentsch, M Pusch - Physiological reviews, 2018 - journals.physiology.org
CLC anion transporters are found in all phyla and form a gene family of eight members in
mammals. Two CLC proteins, each of which completely contains an ion translocation …

Cryo-EM structures of ClC-2 chloride channel reveal the blocking mechanism of its specific inhibitor AK-42

T Ma, L Wang, A Chai, C Liu, W Cui, S Yuan… - Nature …, 2023 - nature.com
ClC-2 transports chloride ions across plasma membranes and plays critical roles in cellular
homeostasis. Its dysfunction is involved in diseases including leukodystrophy and primary …

Revealing the activation pathway for TMEM16A chloride channels from macroscopic currents and kinetic models

JA Contreras-Vite, S Cruz-Rangel… - … -European Journal of …, 2016 - Springer
Abstract TMEM16A (ANO1), the pore-forming subunit of calcium-activated chloride
channels, regulates several physiological and pathophysiological processes such as …

Gating and anion selectivity are reciprocally regulated in TMEM16A (ANO1)

JJ De Jesús-Pérez, AE López-Romero… - Journal of General …, 2022 - rupress.org
Numerous essential physiological processes depend on the TMEM16A-mediated Ca2+-
activated chloride fluxes. Extensive structure–function studies have helped to elucidate the …

Renal chloride channels in relation to sodium chloride transport

J Teulon, G Planelles, FV Sepúlveda… - Comprehensive …, 2011 - Wiley Online Library
The many mechanisms governing NaCl absorption in the diverse parts of the renal tubule
have been largely elucidated, although some of them, as neutral NaCl absorption across the …

CryoEM structures of the human CLC-2 voltage-gated chloride channel reveal a ball-and-chain gating mechanism

M Xu, T Neelands, AS Powers, Y Liu, SD Miller… - Elife, 2024 - elifesciences.org
CLC-2 is a voltage-gated chloride channel that contributes to electrical excitability and ion
homeostasis in many different tissues. Among the nine mammalian CLC homologs, CLC-2 …

Extracellular protons enable activation of the calcium‐dependent chloride channel TMEM16A

S Cruz‐Rangel, JJ De Jesús‐Pérez… - The Journal of …, 2017 - Wiley Online Library
Key points The calcium‐activated chloride channel TMEM16A provides a pathway for
chloride ion movements that are key in preventing polyspermy, allowing fluid secretion …

Regulation of ClC-2 gating by intracellular ATP

G Stölting, G Teodorescu, B Begemann… - … -European Journal of …, 2013 - Springer
ClC-2 is a voltage-dependent chloride channel that activates slowly at voltages negative to
the chloride reversal potential. Adenosine triphosphate (ATP) and other nucleotides have …

Gating the glutamate gate of CLC-2 chloride channel by pore occupancy

JJ De Jesús-Pérez, A Castro-Chong… - Journal of General …, 2016 - rupress.org
CLC-2 channels are dimeric double-barreled chloride channels that open in response to
hyperpolarization. Hyperpolarization activates protopore gates that independently regulate …

ClC-1 and ClC-2 form hetero-dimeric channels with novel protopore functions

G Stölting, M Fischer, C Fahlke - Pflügers Archiv-European Journal of …, 2014 - Springer
CLC-type chloride channels exhibit a unique double-barreled architecture with two
independently functioning ion conduction pathways, the so-called protopores. There exist …