Discovery of CLC transport proteins: cloning, structure, function and pathophysiology

X-exome sequencing of 405 unresolved families identifies seven novel intellectual disability genes

(2015) H Hu et al.   MOLECULAR PSYCHIATRY

Mice with megalencephalic leukoencephalopathy with cysts: A developmental angle

(2014) Mohit Dubey et al.   ANNALS OF NEUROLOGY

GlialCAM, a CLC-2 Cl - Channel Subunit, Activates the Slow Gate of CLC Chloride Channels

(2014) Elena Jeworutzki et al.   BIOPHYSICAL JOURNAL

SWELL1, a Plasma Membrane Protein, Is an Essential Component of Volume-Regulated Anion Channel

(2014) Zhaozhu Qiu et al.   CELL

Transport activity and presence of ClC-7/Ostm1 complex account for different cellular functions

(2014) S. Weinert et al.   EMBO REPORTS

Subclinical leukodystrophy and infertility in a man with a novel homozygous CLCN2 mutation

(2014) D. Di Bella et al.   NEUROLOGY

Vesicular Glutamate Transporters Use Flexible Anion and Cation Binding Sites for Efficient Accumulation of Neurotransmitter

(2014) Julia Preobraschenski et al.   NEURON

Identification of LRRC8 Heteromers as an Essential Component of the Volume-Regulated Anion Channel VRAC

(2014) F. K. Voss et al.   SCIENCE

Involvement of ClC-3 chloride/proton exchangers in controlling glutamatergic synaptic strength in cultured hippocampal neurons

(2014) Raul E. Guzman et al.   Frontiers in Cellular Neuroscience

Disrupting MLC1 and GlialCAM and ClC-2 interactions in leukodystrophy entails glial chloride channel dysfunction

(2014) Maja B. Hoegg-Beiler et al.   Nature Communications

ClC-3 Is an Intracellular Chloride/Proton Exchanger with Large Voltage-Dependent Nonlinear Capacitance

(2013) Raul E. Guzman et al.   ACS Chemical Neuroscience

Characterization of the mouse ClC-K1/Barttin chloride channel

(2013) Sébastien L'Hoste et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Exome sequencing reveals new causal mutations in children with epileptic encephalopathies

(2013) Krishna R. Veeramah et al.   EPILEPSIA

Common Gating of Both CLC Transporter Subunits Underlies Voltage-dependent Activation of the 2Cl−/1H+Exchanger ClC-7/Ostm1

(2013) Carmen F. Ludwig et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Brain white matter oedema due to ClC-2 chloride channel deficiency: an observational analytical study

(2013) Christel Depienne et al.   LANCET NEUROLOGY

Japanese Dent disease has a wider clinical spectrum than Dent disease in Europe/USA: genetic and clinical studies of 86 unrelated patients with low-molecular-weight proteinuria

(2013) Takashi Sekine et al.   NEPHROLOGY DIALYSIS TRANSPLANTATION

Novel brain expression of ClC-1 chloride channels and enrichment of CLCN1 variants in epilepsy

(2013) T. T. Chen et al.   NEUROLOGY

Chloride channel 4 is required for nerve growth factor-induced TrkA signaling and neurite outgrowth in PC12 cells and cortical neurons

(2013) J. Hur et al.   NEUROSCIENCE

Huntington disease skeletal muscle is hyperexcitable owing to chloride and potassium channel dysfunction

(2013) C. W. Waters et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Intracellular β-Nicotinamide Adenine Dinucleotide Inhibits the Skeletal Muscle ClC-1 Chloride Channel

(2012) Brett Bennetts et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

GlialCAM, a Protein Defective in a Leukodystrophy, Serves as a ClC-2 Cl− Channel Auxiliary Subunit

(2012) Elena Jeworutzki et al.   NEURON

Mutant GlialCAM Causes Megalencephalic Leukoencephalopathy with Subcortical Cysts, Benign Familial Macrocephaly, and Macrocephaly with Retardation and Autism

(2011) Tania López-Hernández et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Movement of hClC-1 C-termini during common gating and limits on their cytoplasmic location

(2011) Linlin Ma et al.   BIOCHEMICAL JOURNAL

ClC-7 is a slowly voltage-gated 2Cl−/1H+-exchanger and requires Ostm1 for transport activity

(2011) Lilia Leisle et al.   EMBO JOURNAL

Severe Defects in Absorptive Ion Transport in Distal Colons of Mice That Lack ClC-2 Channels

(2011) Marcelo A. Catalán et al.   GASTROENTEROLOGY

ClC-2 Channels Regulate Neuronal Excitability, Not Intracellular Chloride Levels

(2011) S. Ratte et al.   JOURNAL OF NEUROSCIENCE

Presynaptic CLC-3 determines quantal size of inhibitory transmission in the hippocampus

(2011) Vladimir Riazanski et al.   NATURE NEUROSCIENCE

ClC-3—A Granular Anion Transporter Involved in Insulin Secretion?

(2010) Thomas J. Jentsch et al.   Cell Metabolism

Role of ClC-5 in Renal Endocytosis Is Unique among ClC Exchangers and Does Not Require PY-motif-dependent Ubiquitylation

(2010) Gesa Rickheit et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Sorting Motifs of the Endosomal/Lysosomal CLC Chloride Transporters

(2010) Tobias Stauber et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Late Endosomal ClC-6 Mediates Proton/Chloride Countertransport in Heterologous Plasma Membrane Expression

(2010) Ioana Neagoe et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A cation counterflux supports lysosomal acidification

(2010) Benjamin E. Steinberg et al.   JOURNAL OF CELL BIOLOGY

Distinct Neuropathologic Phenotypes After Disrupting the Chloride Transport Proteins ClC-6 or ClC-7/Ostm1

(2010) Sarah N.R. Pressey et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

ClC-2 Voltage-Gated Channels Constitute Part of the Background Conductance and Assist Chloride Extrusion

(2010) I. Rinke et al.   JOURNAL OF NEUROSCIENCE

Design, function and structure of a monomeric ClC transporter

(2010) Janice L. Robertson et al.   NATURE

Regulation of fast-spiking basket cell synapses by the chloride channel ClC-2

(2010) Csaba Földy et al.   NATURE NEUROSCIENCE

Lysosomal Pathology and Osteopetrosis upon Loss of H+-Driven Lysosomal Cl- Accumulation

(2010) S. Weinert et al.   SCIENCE

Structure of a Eukaryotic CLC Transporter Defines an Intermediate State in the Transport Cycle

(2010) Liang Feng et al.   SCIENCE

Endosomal Chloride-Proton Exchange Rather Than Chloride Conductance Is Crucial for Renal Endocytosis

(2010) G. Novarino et al.   SCIENCE

Molecular Basis of DFNB73: Mutations of BSND Can Cause Nonsyndromic Deafness or Bartter Syndrome

(2009) Saima Riazuddin et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Lysosomal degradation of endocytosed proteins depends on the chloride transport protein ClC-7

(2009) Lena Wartosch et al.   FASEB JOURNAL

Residues Important for Nitrate/Proton Coupling in Plant and Mammalian CLC Transporters

(2009) Eun-Yeong Bergsdorf et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Retraction Note to: Retraction: Mutations in CLCN2 encoding a voltage-gated chloride channel are associated with idiopathic generalized epilepsies

(2009) Karsten Haug et al.   NATURE GENETICS

No evidence for a role of CLCN2 variants in idiopathic generalized epilepsy

(2009) María I Niemeyer et al.   NATURE GENETICS

A chloride conductance in VGLUT1 underlies maximal glutamate loading into synaptic vesicles

(2009) Stephan Schenck et al.   NATURE NEUROSCIENCE

CLC Chloride Channels and Transporters: From Genes to Protein Structure, Pathology and Physiology

(2008) Thomas J. Jentsch   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

Endocochlear potential depends on Cl− channels: mechanism underlying deafness in Bartter syndrome IV

(2008) Gesa Rickheit et al.   EMBO JOURNAL

ATP Inhibition of CLC-1 Is Controlled by Oxidation and Reduction

(2008) Xiao-Dong Zhang et al.   JOURNAL OF GENERAL PHYSIOLOGY

Role of the Vesicular Chloride Transporter ClC-3 in Neuroendocrine Tissue

(2008) T. Maritzen et al.   JOURNAL OF NEUROSCIENCE

The Cl-/H+ antiporter ClC-7 is the primary chloride permeation pathway in lysosomes

(2008) Austin R. Graves et al.   NATURE

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

(2008) Jiří Lísal et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Overexpression of CLC-3 in HEK293T cells yields novel currents that are pH dependent

(2007) James J. Matsuda et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY