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 …

CLC chloride channels and transporters: from genes to protein structure, pathology and physiology

TJ Jentsch - Critical reviews in biochemistry and molecular biology, 2008 - Taylor & Francis
ABSTRACT CLC genes are expressed in species from bacteria to human and encode Cl−-
channels or Cl−/H+-exchangers. CLC proteins assemble to dimers, with each monomer …

Functional and clinical studies reveal pathophysiological complexity of CLCN4-related neurodevelopmental condition

EE Palmer, M Pusch, A Picollo, C Forwood… - Molecular …, 2023 - nature.com
Missense and truncating variants in the X-chromosome-linked CLCN4 gene, resulting in
reduced or complete loss-of-function (LOF) of the encoded chloride/proton exchanger ClC-4 …

[HTML][HTML] CLC channels and transporters: proteins with borderline personalities

A Accardi, A Picollo - Biochimica et Biophysica Acta (BBA)-Biomembranes, 2010 - Elsevier
Controlled chloride movement across membranes is essential for a variety of physiological
processes ranging from salt homeostasis in the kidneys to acidification of cellular …

Uncoupling and Turnover in a Cl/H+ Exchange Transporter

M Walden, A Accardi, F Wu, C Xu, C Williams… - The Journal of general …, 2007 - rupress.org
The CLC-family protein CLC-ec1, a bacterial homologue of known structure,
stoichiometrically exchanges two Cl− for one H+ via an unknown membrane transport …

CLC-0 and CFTR: chloride channels evolved from transporters

TY Chen, TC Hwang - Physiological reviews, 2008 - journals.physiology.org
CLC-0 and cystic fibrosis transmembrane conductance regulator (CFTR) Cl− channels play
important roles in Cl− transport across cell membranes. These two proteins belong to …

Structure and gating of CLC channels and exchangers

A Accardi - The Journal of physiology, 2015 - Wiley Online Library
Since their serendipitous discovery the CLC family of Cl− transporting proteins has been a
never ending source of surprises. From their double‐barrelled architecture to their complex …

Molecular determinants underlying volume‐regulated anion channel subunit‐dependent oxidation sensitivity

S Bertelli, P Zuccolini, P Gavazzo… - The Journal of …, 2022 - Wiley Online Library
The volume‐regulated anion channel (VRAC) is formed by LRRC8 subunits. Besides their
role in the maintenance of cell homeostasis, VRACs are critically involved in oxidative stress …

CLC chloride channels and transporters: a biophysical and physiological perspective

G Zifarelli, M Pusch - Reviews of physiology, biochemistry and …, 2007 - Springer
Chloride-transporting proteins play fundamental roles in many tissues in the plasma
membrane as well as in intracellular membranes. They have received increasing attention in …

The ClC-0 chloride channel is a 'broken' Cl/H+ antiporter

J Lísal, M Maduke - Nature structural & molecular biology, 2008 - nature.com
Ion channels have historically been viewed as distinct from secondary active transporters.
However, the recent discovery that the CLC'chloride channel'family is made up of both …