Status of Fluid and Electrolyte Absorption in Cystic Fibrosis

Reddy, M. M.; Stutts, M. Jackson

doi:10.1101/cshperspect.a009555

Cited by 14 publications

(8 citation statements)

References 157 publications

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“…Eccrine sweat glands are one of the simplest exocrine gland systems [46]. Each eccrine sweat gland unit consists of two portions, the secretory coil and the duct, which differ in morphology and function (Figure 2A) [55, 56].…”

Section: Mechanism Of Salt and Fluid Secretion In Eccrine Sweat Glmentioning

confidence: 99%

Regulation of epithelial ion transport in exocrine glands by store-operated Ca2+ entry

Concepcion

Feske

2017

Cell Calcium

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Store-operated Ca2+ entry (SOCE) is a conserved mechanism of Ca2+ influx that regulates Ca2+ signaling in many cell types. SOCE is activated by depletion of endoplasmic reticulum (ER) Ca2+ stores in response to physiological agonist stimulation. After it was first postulated by J.W. Putney Jr. in 1986, SOCE has been described in a large number of non-excitable cell types including secretory cells of different exocrine glands. Here we discuss the mechanisms by which SOCE controls salt and fluid secretion in exocrine glands, with a special focus on eccrine sweat glands. In sweat glands, SOCE plays an important, non-redundant role in regulating the function of Ca2+-activated Cl− channels (CaCC), Cl− secretion and sweat production. In the absence of key regulators of SOCE such as the CRAC channel pore subunit ORAI1 and its activator STIM1, the Ca2+-activated chloride channel TMEM16A is inactive and fails to secrete Cl−, resulting in anhidrosis in mice and human patients.

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Section: Mechanism Of Salt and Fluid Secretion In Eccrine Sweat Glmentioning

confidence: 99%

Regulation of epithelial ion transport in exocrine glands by store-operated Ca2+ entry

Concepcion

Feske

2017

Cell Calcium

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“…ZO-1 also provides a link between airway barrier function and the Cystic Fibrosis Transmembrane Regulator (CFTR) chloride channel [82, 83], which is important for maintenance of airway surface liquid composition [5, 84, 85], mucociliary clearance [86, 87], and innate immune responses. [4, 88, 89].…”

Section: Claudin Structure and Composition In Tight Junctionsmentioning

confidence: 99%

Claudins: Gatekeepers of lung epithelial function

Schlingmann

Molina

Koval

2015

Seminars in Cell & Developmental Biology

149

108

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The lung must maintain a proper barrier between airspaces and fluid filled tissues in order to maintain lung fluid balance. Central to maintaining lung fluid balance are epithelial cells which create a barrier to water and solutes. The barrier function of these cells is mainly provided by tight junction proteins known as claudins. Epithelial barrier function varies depending on the different needs within the segments of the respiratory tree. In the lower airways, fluid is required to maintain mucociliary clearance, whereas in the terminal alveolar airspaces a thin layer of surfactant enriched fluid lowers surface tension to prevent airspace collapse and is critical for gas exchange. As the epithelial cells within the segments of the respiratory tree differ, the composition of claudins found in these epithelial cells is also different. Among these differences is claudin-18 which is uniquely expressed by the alveolar epithelial cells. Other claudins, notably claudin-4 and claudin-7, are more ubiquitously expressed throughout the respiratory epithelium. Claudin-5 is expressed by both pulmonary epithelial and endothelial cells. Based on in vitro and in vivo model systems and histologic analysis of lungs from human patients, roles for specific claudins in maintaining barrier function and protecting the lung from the effects of acute injury and disease are being identified. One surprising finding is that claudin-18 and claudin-4 control lung cell phenotype and inflammation beyond simply maintaining a selective paracellular permeability barrier. This suggests claudins have more nuanced roles for the control of airway and alveolar physiology in the healthy and diseased lung.

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“…In terms of permeability properties, epithelia are classified into two groups as leaky- and tight-epithelia (Fischbarg, 2010; Reddy and Stutts, 2013). Leaky epithelia are located generally in an isoosmotic environment as in the small intestine and proximal kidney tubules and are highly permeable to water.…”

Section: Introductionmentioning

confidence: 99%

“…Leaky epithelia are located generally in an isoosmotic environment as in the small intestine and proximal kidney tubules and are highly permeable to water. In contrast to leaky epithelia, the cells in tight epithelia are connected by complex tight junctions that reduce the permeability of the epithelia (Capaldo et al, 2014; Reddy and Stutts, 2013). …”

Section: Introductionmentioning

confidence: 99%

Epithelial sodium channel (ENaC) family: Phylogeny, structure–function, tissue distribution, and associated inherited diseases

Hanukoglu

2016

Gene

337

345

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The epithelial sodium channel (ENaC) is composed of three homologous subunits and allows the flow of Na+ ions across high resistance epithelia, maintaining body salt and water homeostasis. ENaC dependent reabsorption of Na+ in the kidney tubules regulates extracellular fluid (ECF) volume and blood pressure by modulating osmolarity. In multi-ciliated cells, ENaC is located in cilia and plays an essential role in the regulation of epithelial surface liquid volume necessary for cilial transport of mucus and gametes in the respiratory and reproductive tracts respectively. The subunits that form ENaC (named as alpha, beta, gamma and delta, encoded by genes SCNN1A, SCNN1B, SCNN1G, and SCNN1D) are members of the ENaC/Degenerin superfamily. The earliest appearance of ENaC orthologs is in the genomes of the most ancient vertebrate taxon, Cyclostomata (jawless vertebrates) including lampreys, followed by earliest representatives of Gnathostomata (jawed vertebrates) including cartilaginous sharks. Among Euteleostomi (bony vertebrates), Actinopterygii (ray finned-fishes) branch has lost ENaC genes. Yet, most animals in the Sarcopterygii (lobe-finned fish) branch including Tetrapoda, amphibians and amniotes (lizards, crocodiles, birds, and mammals), have four ENaC paralogs. We compared the sequences of ENaC orthologs from 20 species and established criteria for the identification of ENaC orthologs and paralogs, and their distinction from other members of the ENaC/Degenerin superfamily, especially ASIC family. Differences between ENaCs and ASICs are summarized in view of their physiological functions and tissue distributions. Structural motifs that are conserved throughout vertebrate ENaCs are highlighted. We also present a comparative overview of the genotype-phenotype relationships in inherited diseases associated with ENaC mutations, including multisystem pseudohypoaldosteronism (PHA1B), Liddle syndrome, cystic fibrosis-like disease and essential hypertension.

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Status of Fluid and Electrolyte Absorption in Cystic Fibrosis

Cited by 14 publications

References 157 publications

Regulation of epithelial ion transport in exocrine glands by store-operated Ca2+ entry

Regulation of epithelial ion transport in exocrine glands by store-operated Ca2+ entry

Claudins: Gatekeepers of lung epithelial function

Epithelial sodium channel (ENaC) family: Phylogeny, structure–function, tissue distribution, and associated inherited diseases

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