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CLC Chloride Channels and Transporters: Structure, Function, Physiology, and Disease

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CLC Chloride Channels and Transporters: Structure, Function, Physiology, and Disease Repository of the Max Delbrück Center for Molecular Medicine (MDC) in the Helmholtz Association https://edoc.mdc-berlin.de/17475/ CLC chloride channels and transporters: structure, function, physiology, and disease Jentsch T.J., Pusch M. This is a copy of the accepted manuscript, as originally published online ahead of print by the American Physiological Society. The original article has been published in final edited form in: Physiological Reviews 2018 JUL ; 98 (3): 1493-1590 2019 MAY 30 (first published online) Doi: 10.1152/physrev.00047.2017 Publisher: American Physiological Society Copyright © 2018 the American Physiological Society CLC Chloride Channels and Transporters - Structure, Function, Physiology and Disease Thomas J. Jentsch1 and Michael Pusch2 1 Leibniz-Forschungsinstitut für Molekulare Pharmakologie (FMP), and Max-Delbrück-Centrum für Molekulare Medizin, 13125 Berlin, Germany 2 Istituto di Biofisica, Consiglio Nazionale delle Ricerche, 16149 Genova, Italy 1 Abstract CLC anion transporters are found in all phylae and form a gene family of eight members in mammals. Two CLC proteins, each of which completely contains an ion translocation parthway, assemble to homo- or heteromeric dimers that sometimes require accessory β-subunits for function. CLC proteins come in two flavors: Anion channels and anion/proton exchangers. Structures of these two CLC protein classes are surprisingly similar. Extensive structure-function analysis identified residues involved in ion permeation, anion-proton coupling and gating and led to attractive biophysical models. In mammals, ClC-1, -2, -Ka/-Kb are plasma membrane Cl- channels, whereas ClC-3 through ClC-7 are 2Cl-/H+-exchangers in endo-lysosomal membranes. Biological roles of CLCs were mostly studied in mammals, but also in plants and model organisms like yeast and C. elegans. CLC Cl- channels have roles in the control of electrical excitability, extra- and intracellular ion homeostasis and transepithelial transport, whereas anion/proton exchangers influence vesicular ion composition and impinge on endocytosis and lysosomal function. The surprisingly diverse roles of CLCs are highlighted by human and mouse disorders elicited by mutations in their genes. These pathologies include neurodegeneration, leukodystophy, mental retardation, deafness, blindness, myotonia, hyperaldosteronism, renal salt loss, proteinuria, kidney stones, male infertility and osteopetrosis. In this review, emphasis is laid on biophysical structure-function analysis and on the cell biological and organismal roles of mammalian CLCs and their role in disease. I. INTRODUCTION II. BIOPHYSICAL PROPERTIES OF CLC PROTEINS III. CELL BIOLOGY, PHYSIOLOGY AND PATHOLOGY OF MAMMALIAN CLC PROTEINS IV. CLC PLASMA MEMBRANE CHLORIDE CHANNELS V. ClC-1 – THE SKELETAL MUSCLE CL- CHANNEL MUTATED IN MYOTONIA VI. ClC-2 – A WIDELY EXPRESSED CL- CHANNEL INVOLVED IN VARIOUS PROCESSES VII. ClC-KA/KB-BARTTIN CHANNELS – HIGHLY CELL-SPECIFIC CL- CHANNELS MUTATED IN CERTAIN FORMS OF KIDNEY DISEASE AND DEAFNESS VIII. VESICULAR 2CL-/H+ EXCHANGERS IX. ClC-3 – A WIDELY EXPRESSED, MAINLY ENDOSOMAL CL-/H+ EXCHANGER X. ClC-4 – A VESICULAR CL-/H+ EXCHANGER WITH SLOWLY EMERGING ROLES XI. ClC-5 – A KIDNEY-SPECIFIC CL-/H+ ANTIPORTER INVOLVED IN RENAL ENDOCYTOSIS XII. ClC-6 – A LATE ENDOSOMAL CL-/H+ EXCHANGER OF NEURONS XIII. ClC-7 – A LYSOSOMAL CL-/H+ ANTIPORTER MUTATED IN OSTEOPETROSIS AND NEURODEGENERATION XIV. CLCs IN OTHER ORGANISMS XV. OUTLOOK 2 I. INTRODUCTION Chloride is the most abundant anion and serves many different biological roles. As a counterion for Na+ and K+, chloride ensures electroneutrality both under steady state and during transport across cellular membranes, as exemplified by transepithelial transport and acidification of intracellular vesicles. Owed to its high concentration, it serves, together with positively charged counterions, as important osmolyte to drive water across cellular membranes in cell volume regulation and transepithelial secretion or absorption of water. Chloride may also have ‘chemical’ roles by binding to proteins and thereby modifying their function. Examples are given by the lysosomal enzyme cathepsin C, the activity of which is modulated by the binding of chloride to the protein (138) and WNK protein kinases (616, 646, 830), but many other proteins are known to be chloride-sensitive (207). Chloride transport across cellular membranes generates electrical currents if its transport is not strictly coupled to that of other ions, such as + - + + - - - - in ‘electroneutral’ K Cl or Na K 2Cl cotransporters or Cl /HCO3 exchangers. Cl channels can therefore change the voltage across the plasma membrane and thereby influence the electrical excitability of neurons, muscle, and endocrine cells (310, 423, 477, 572, 702). Cl- channels might also influence the voltage of intracellular organelles, but most likely the main Cl- transporters of endosomes and lysosomes are 2Cl-/H+-exchangers of the CLC gene family, which encompasses both these exchangers and Cl- channels. These exchangers also generate currents (are ‘electrogenic’), but, as discussed below, coupling of Cl- to H+ transport has additional important implications for their biological roles. The direction of Cl- transport through channels is entirely determined by the transmembrane gradient of the electrochemical potential, which is given by the difference in Cl- concentration and the transmembrane voltage according to the Nernst equation. Whereas in mammals the serum concentration of Cl- is universally high (~120 mM) and intracellular chloride - - concentration ([Cl ]int) much lower, different cell types display a wide range of [Cl ]int (from ~5- - 10 mM in most mature neurons up to ~40 mM in epithelial cells). This large difference in [Cl ]int is mainly due to differential expression of secondarily active Cl- transporters that either increase + + - - - + - (e.g. Na K 2Cl cotransporters or Cl /HCO3 exchangers) or decrease (like K Cl cotransporters) - - [Cl ]int (201, 889). Hence opening of Cl channels may either hyperpolarize or depolarize the plasma membrane. In stark contrast, opening of Na+ and Ca2+ channels will almost always depolarize the cell owing to the large inwardly-directed ion gradient and the inside-negative voltage. For a long time, the study of chloride channels had been neglected. The interest focused on voltage- or ligand-gated cation channels that underlie action potential conduction and synaptic transmission (343). In fact, Cl- channels were often considered a nuisance and their currents were often eliminated when studying cation channels. Notable exceptions are the large chloride conductance of skeletal muscle, that amounts to about 80% of the resting conductance 3 (83, 364, 477, 617), and inhibitory GABA and glycine receptor anion channels (300, 572, 752). In the 1980’s the application of the patch clamp technique revealed the presence of cation and anion channels in practically all cells (95, 343, 776). The first voltage-gated chloride channel to be molecularly identified was ClC-0, the founding member of the CLC gene family (393). Thomas Jentsch and colleagues cloned it from the electric organ of the marine ray Torpedo which is a rich source for ion channels that are needed to generate the large currents which these animals use to stun their prey. Besides the nicotinic acetylcholine receptor cation channel, which also was first cloned from this organ (596), the Torpedo electroplax contained a peculiar chloride channel which had been discovered by Chris Miller upon reconstituting electric organ membrane proteins into lipid bilayers (905), in this way establishing the first high resolution single channel recordings from lipid bilayers (548). Detailed biophysical analysis by Miller and colleagues strongly suggested that the channel contained two largely independent pores (324, 548, 549), a prescient conclusion amply confirmed after its molecular identification (211, 491, 492, 543, 897). Thomas Jentsch at first tried to identify the channel protein by its ability to covalently bind DIDS (388), which Chris Miller had shown to irreversibly inhibit the channel at micromolar concentrations (549). Following the failure of his approach (388), Jentsch and colleagues finally identified the channel using a painstaking combination of hybrid depletion and functional expression in Xenopus oocytes (393). After having identified the mammalian skeletal muscle chloride channel, which they named ClC-1 (for Cl- Channel 1), they proposed to name the prototype Torpedo channel ClC-0 (807). Personal accounts of Jentsch’s and Miller’s initial discoveries can be found in two recent reviews (385, 547). Using homology based methods, first in wet-lab experiments using hybridization of cDNA libraries with radioactive probes (807, 808, 832) and later in silico once large-scale DNA sequencing took off, CLC proteins (FOOTNOTE) were subsequently identified in all phyla, with nine human CLC genes (384, 385) (Fig. 1). Apart from the skeletal muscle ClC-1 channel, whose function was known before its molecular identification (807), the function of the remaining CLCs was unknown. The study of their biochemical and biophysical properties and of their physiological role and their involvement in human genetic diseases led to the clarification and discovery of many biological and pathological processes (384, 802). A particularly important discovery was that several CLCs reside in the endo/lysosomal system (307, 429) and that these intracellular CLCs are actually not chloride
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