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https://www.readbyqxmd.com/read/27903760/interactions-between-intersubunit-transmembrane-domains-regulate-the-chaperone-dependent-degradation-of-an-oligomeric-membrane-protein
#1
Teresa M Buck, Alexa S Jordahl, Megan E Yates, Mike Preston, Emily Cook, Thomas R Kleyman, Jeffrey L Brodsky
The Epithelial Sodium Channel (ENaC) in kidney regulates blood pressure through control of sodium and volume homeostasis, and in lung ENaC regulates the volume of airway and alveolar fluids.  ENaC is a heterotrimer of homologous α-, β-, and γ-subunits, and assembles in the Endoplasmic Reticulum (ER) before it traffics and functions at the plasma membrane. Improperly folded or orphaned ENaC subunits are subject to ER quality control and targeted for ER associated degradation (ERAD). We previously established that a conserved, ER lumenal, molecular chaperone, Lhs1/GRP170, selects αENaC, but not β- or γENaC, for degradation when the ENaC subunits were individually expressed...
November 30, 2016: Biochemical Journal
https://www.readbyqxmd.com/read/27900368/whole-exome-sequencing-reveals-an-inherited-r566x-mutation-of-the-epithelial-sodium-channel-%C3%AE-subunit-in-a-case-of-early-onset-phenotype-of-liddle-syndrome
#2
Linda M Polfus, Eric Boerwinkle, Richard A Gibbs, Ginger Metcalf, Donna Muzny, Narayanan Veeraraghavan, Megan Grove, Sanjay Shete, Stephanie Wallace, Dianna Milewicz, Neil Hanchard, James R Lupski, Syed Shahrukh Hashmi, Monesha Gupta-Malhotra
To comprehensively evaluate a European-American child with severe hypertension, whole-exome sequencing (WES) was performed on the child and parents, which identified causal variation of the proband's early-onset disease. The proband's hypertension was resistant to treatment, requiring a multiple drug regimen including amiloride, spironolactone, and hydrochlorothiazide. We suspected a monogenic form of hypertension because of the persistent hypokalemia with low plasma levels of renin and aldosterone. To address this, we focused on rare functional variants and indels, and performed gene-based tests incorporating linkage scores and allele frequency and filtered on deleterious functional mutations...
November 2016: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/27895120/ankyrin-g-expression-regulates-apical-delivery-of-the-epithelial-sodium-channel-enac
#3
Christine A Klemens, Robert S Edinger, Lindsay Kightlinger, Xiaoning Liu, Michael B Butterworth
The epithelial sodium channel (ENaC) is the limiting entry point for Na(+) reabsorption in the distal kidney nephron and is regulated by numerous hormones, including the mineralocorticoid hormone aldosterone. Previously we identified ankyrin G (AnkG), a cytoskeletal protein involved in vesicular transport, as a novel aldosterone-induced protein that can alter Na(+) transport in mouse cortical collecting duct (mCCD) cells. However, the mechanisms underlying AnkG's regulation of Na(+) transport were unknown. Here we report that AnkG expression directly regulates Na(+) transport by altering ENaC activity in the apical membrane...
November 28, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27867979/na-homeostasis-by-epithelial-na-channel-enac-and-nax-channel-nax-cooperation-of-enac-and-nax
#4
EDITORIAL
Yoshinori Marunaka, Rie Marunaka, Hongxin Sun, Toshiro Yamamoto, Narisato Kanamura, Akiyuki Taruno
No abstract text is available yet for this article.
October 2016: Annals of Translational Medicine
https://www.readbyqxmd.com/read/27835672/the-expression-of-water-and-ion-channels-in-diffuse-alveolar-damage-is-not-dependent-on-dad-etiology
#5
Ruy Camargo Pires-Neto, Fabiola Del Carlo Bernardi, Priscila Alves de Araujo, Thais Mauad, Marisa Dolhnikoff
INTRODUCTION: Aquaporins and ion channels are membrane proteins that facilitate the rapid movement of water and solutes across biological membranes. Experimental and in vitro studies reported that the function of these channels and pulmonary edema resolution are impaired in acute lung injury (ALI). Although current evidence indicates that alveolar fluid clearance is impaired in patients with ALI/diffuse alveolar damage (DAD), few human studies have addressed the alterations in pulmonary channels in this clinical condition...
2016: PloS One
https://www.readbyqxmd.com/read/27833542/adaptive-automation-triggered-by-eeg-based-mental-workload-index-a-passive-brain-computer-interface-application-in-realistic-air-traffic-control-environment
#6
Pietro Aricò, Gianluca Borghini, Gianluca Di Flumeri, Alfredo Colosimo, Stefano Bonelli, Alessia Golfetti, Simone Pozzi, Jean-Paul Imbert, Géraud Granger, Raïlane Benhacene, Fabio Babiloni
Adaptive Automation (AA) is a promising approach to keep the task workload demand within appropriate levels in order to avoid both the under- and over-load conditions, hence enhancing the overall performance and safety of the human-machine system. The main issue on the use of AA is how to trigger the AA solutions without affecting the operative task. In this regard, passive Brain-Computer Interface (pBCI) systems are a good candidate to activate automation, since they are able to gather information about the covert behavior (e...
2016: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/27830759/marked-increases-in-mucociliary-clearance-produced-by-synergistic-secretory-agonists-or-inhibition-of-the-epithelial-sodium-channel
#7
Nam Soo Joo, Jin Hyeok Jeong, Hyung-Ju Cho, Jeffrey J Wine
Mucociliary clearance (MCC) is a critical host innate defense mechanism in airways, and it is impaired in cystic fibrosis (CF) and other obstructive lung diseases. Epithelial fluid secretion and absorption modify MCC velocity (MCCV). We tested the hypotheses that inhibiting fluid absorption accelerates MCCV, whereas inhibiting fluid secretion decelerates it. In airways, ENaC is mainly responsible for fluid absorption, while anion channels, including CFTR and Ca(2+)-activated chloride channels mediate anion/fluid secretion...
November 10, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27825168/activation-of-sgk1-in-endometrial-epithelial-cells-in-response-to-pi3k-akt-inhibition-impairs-embryo-implantation
#8
Madhuri S Salker, Jennifer H Steel, Zohreh Hosseinzadeh, Jaya Nautiyal, Zoe Webster, Yogesh Singh, Sara Brucker, Florian Lang, Jan J Brosens
BACKGROUND: Serum & Glucocorticoid Regulated Kinase 1 (SGK1) plays a fundamental role in ion and solute transport processes in epithelia. In the endometrium, down-regulation of SGK1 during the window of receptivity facilitates embryo implantation whereas expression of a constitutively active mutant in the murine uterus blocks implantation. METHODS/RESULTS: Here, we report that treatment of endometrial epithelial cells with specific inhibitors of the phosphoinositide 3-kinase (PI3K)/AKT activity pathway results in reciprocal activation of SGK1...
2016: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/27815150/solnatide-demonstrates-profound-therapeutic-activity-in-a-rat-model-of-pulmonary-edema-induced-by-acute-hypobaric-hypoxia-and-exercise
#9
Qiquan Zhou, Dong Wang, Yunsheng Liu, Xiaohong Yang, Rudolf Lucas, Bernhard Fischer
BACKGROUND: The synthetic peptide solnatide is a novel pharmacological agent that reduces extravascular lung water, blunts reactive oxygen species production and improves lung function due to its ability to directly activate the epithelial sodium channel ENaC. We aimed to investigate the effect of solnatide in pulmonary edema induced by acute hypobaric hypoxia and exercise in rats, which is considered a model for high altitude pulmonary edema. METHODS: Sprague-Dawley rats were assigned to low altitude control and eight treatment groups...
November 1, 2016: Chest
https://www.readbyqxmd.com/read/27814452/water-permeability-adjusts-resorption-in-lung-epithelia-to-increased-apical-surface-liquid-volumes
#10
Hanna Schmidt, Christiane Michel, Peter Braubach, Michael Fauler, Daniel Neubauer, Kristin E Thompson, Manfred Frick, Boris Mizaikoff, Paul Dietl, Oliver H Wittekindt
The apical surface liquid layer (ASL) covers the airways and forms a first line of defense against pathogens. Maintenance of ASL volume by airway epithelia is essential for maintaining lung function. The proteolytic activation of epithelial Na(+) channels (ENaC) is believed to be the dominating mechanism to cope with increases in ASL volumes. Alternative mechanisms, in particular increases in epithelial water permeability (Posm), have so far been regarded as rather less important. However, most studies mainly addressed immediate effects upon apical volume expansion (AVE) and increases in ASL...
November 4, 2016: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/27804084/ion-transport-its-regulation-in-the-endolymphatic-sac-suggestions-for-clinical-aspects-of-meniere-s-disease
#11
REVIEW
Nozomu Mori, Takenori Miyashita, Ryuhei Inamoto, Ai Matsubara, Terushige Mori, Kosuke Akiyama, Hiroshi Hoshikawa
Ion transport and its regulation in the endolymphatic sac (ES) are reviewed on the basis of recent lines of evidence. The morphological and physiological findings demonstrate that epithelial cells in the intermediate portion of the ES are more functional in ion transport than those in the other portions. Several ion channels, ion transporters, ion exchangers, and so on have been reported to be present in epithelial cells of ES intermediate portion. An imaging study has shown that mitochondria-rich cells in the ES intermediate portion have a higher activity of Na(+), K(+)-ATPase and a higher Na(+) permeability than other type of cells, implying that molecules related to Na(+) transport, such as epithelial sodium channel (ENaC), Na(+)-K(+)-2Cl(-) cotransporter 2 (NKCC2) and thiazide-sensitive Na(+)-Cl(-) cotransporter (NCC), may be present in mitochondria-rich cells...
November 1, 2016: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/27802315/exosomal-gapdh-from-proximal-tubule-cells-regulate-enac-activity
#12
Kishore Kumar Jella, Ling Yu, Qiang Yue, Daniel Friedman, Billie J Duke, Abdel A Alli
Exosomes are nanometer-scale, cell-derived vesicles that contain various molecules including nucleic acids, proteins, and lipids. These vesicles can release their cargo into adjacent or distant cells and mediate intercellular communication and cellular function. Here we examined the regulation of epithelial sodium channels in mpkCCD cells and distal tubule Xenopus 2F3 cells by exosomes isolated from proximal tubule LLC-PK1 cells. Cultured mpkCCD cells were stained with CTX coupled to a green fluorophore in order to label the cell membranes and freshly isolated exosomes from LLC-PK1 cells were labeled with the red lipophilic dye PKH26 in order to visualize uptake of exosomes into the cells...
2016: PloS One
https://www.readbyqxmd.com/read/27797949/complex-evolutionary-dynamics-of-massively-expanded-chemosensory-receptor-families-in-an-extreme-generalist-chelicerate-herbivore
#13
Phuong Cao Thi Ngoc, Robert Greenhalgh, Wannes Dermauw, Stephane Rombauts, Sabina Bajda, Vladimir Zhurov, Miodrag Grbić, Yves Van de Peer, Thomas Van Leeuwen, Pierre Rouzé, Richard M Clark
While mechanisms to detoxify plant produced, anti-herbivore compounds have been associated with plant host use by herbivores, less is known about the role of chemosensory perception in their life histories. This is especially true for generalists, including chelicerate herbivores that evolved herbivory independently from the more studied insect lineages. To shed light on chemosensory perception in a generalist herbivore, we characterized the chemosensory receptors (CRs) of the chelicerate two-spotted spider mite, Tetranychus urticae, an extreme generalist...
October 26, 2016: Genome Biology and Evolution
https://www.readbyqxmd.com/read/27789743/channel-transporter-complexes-an-emerging-theme-in-cell-signaling
#14
Geoffrey W Abbott
In a recent edition of Biochemical Journal, Mistry and colleagues described the discovery of a novel protein complex, formed from the epithelial sodium channel (ENaC) and the sodium chloride cotransporter (NCC) [Biochem. J. (2016) Hoover, S.R]. The importance of these two proteins in the regulation of salt balance and blood pressure has long been known, as has their overlapping expression in the distal convoluted tubule of the kidney. The new study by Mistry et al. now demonstrates their physical interaction in the kidney and when heterologously co-expressed...
November 1, 2016: Biochemical Journal
https://www.readbyqxmd.com/read/27788793/tissue-expression-of-aquaporin-2-is-correlated-to-urine-output-and-allograft-function-in-sensitized-kidney-transplant-patients
#15
K Klein, S Rübenacker, S M Schaefer, L P Kihm, V Schwenger, S Macher-Goeppinger, P Schemmer, C Süsald, M Zeier, C Morath, L E Becker
BACKGROUND: Salt and water disturbances often occur during acute kidney allograft dysfunction that contribute to graft failure, but this condition has been poorly investigated in the alloreactivity setting. We evaluated the tissue expression of aquaporins (AQP1 and AQP2) and the epithelial sodium channel (ENAC) in kidney biopsy specimens from sensitized kidney transplant recipients. METHODS: Eighty-six biopsy specimens from 33 sensitized patients were divided into 3 groups according to clinical context: time-zero (n = 9), protocol (n = 9), and indication (n = 68)...
October 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27780255/an-optimised-human-cell-culture-model-for-alveolar-epithelial-transport
#16
Hui Ren, Nigel P Birch, Vinod Suresh
Robust and reproducible in vitro models are required for investigating the pathways involved in fluid homeostasis in the human alveolar epithelium. We performed functional and phenotypic characterisation of ion transport in the human pulmonary epithelial cell lines NCI-H441 and A549 to determine their similarity to primary human alveolar type II cells. NCI-H441 cells exhibited high expression of junctional proteins ZO-1, and E-cadherin, seal-forming claudin-3, -4, -5 and Na+-K+-ATPase while A549 cells exhibited high expression of pore-forming claudin-2...
2016: PloS One
https://www.readbyqxmd.com/read/27762337/formaldehyde-impairs-transepithelial-sodium-transport
#17
Yong Cui, Huiming Li, Sihui Wu, Runzhen Zhao, Deyi Du, Yan Ding, Hongguang Nie, Hong-Long Ji
Unsaturated oxidative formaldehyde is a noxious aldehyde in cigarette smoke that causes edematous acute lung injury. However, the mechanistic effects of formaldehyde on lung fluid transport are still poorly understood. We examined how formaldehyde regulates human epithelial sodium channels (ENaC) in H441 and expressed in Xenopus oocytes and exposed mice in vivo. Our results showed that formaldehyde reduced mouse transalveolar fluid clearance in vivo. Formaldehyde caused a dose-dependent inhibition of amiloride-sensitive short-circuit Na(+) currents in H441 monolayers and of αβγ-ENaC channel activity in oocytes...
October 20, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27760755/ca2-permeability-and-na-conductance-in-cellular-toxicity-caused-by-hyperactive-deg-enac-channels
#18
Cristina Matthewman, Tyne W Miller-Fleming, David M Miller, Laura Bianchi
Hyperactivated DEG/ENaC channels cause neuronal death mediated by intracellular Ca(2+) overload. Mammalian ASIC1a channels and MEC-4(d) neurotoxic channels in Caenorhabditis elegans both conduct Na(+) and Ca(2+), raising the possibility that direct Ca(2+) influx through these channels contributes to intracellular Ca(2+) overload. However, we showed that the homologous C. elegans DEG/ENaC channel UNC-8(d) is not Ca(2+) permeable, yet it is neurotoxic, suggesting that Na(+) influx is sufficient to induce cell death...
December 1, 2016: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/27754097/os-21-02-connecting-tubule-glomerular-feedback-ctgf-in-renal-hemodynamics-and-blood-pressure-regulation-after-unilateral-nephrectomy-unx
#19
Cesar Romero, Sumit Monu, Glauber Cabral, Robert Knight, Oscar Carretero
OBJECTIVE: Renal hemodynamics is critical for regulation of glomerular filtration (GFR), sodium excretion and blood pressure (BP), and it depends on myogenic response, tubuloglomerular feedback (TGF) and connecting tubule-glomerular feedback (CTGF). CTGF dilates afferent arteriole in response to high sodium in connecting tubule (CNT), counteracting and resetting TGF; and increasing the plasma flow and glomerular pressure favoring sodium excretion. CTGF is initiated by epithelial sodium channel (ENaC) activation in CNT and inhibited by ENaC blocker Benzamil...
September 2016: Journal of Hypertension
https://www.readbyqxmd.com/read/27753817/ssa-05-4-differences-in-management-of-hypertension-between-ethnic-groups
#20
Brian Rayner
Although the consequences of hypertension are universal, Blacks (African Americans or Indigenous Africans) have been the subject of a differential approach to causation, outcome and treatment. Blacks have a greater propensity to salt sensitivity and suppressed plasma renin suggesting a predisposition to Na retention by the kidney. As a result blood pressure (BP) response to diuretics and amlodipine is better than inhibitors of the renin-angiotensin-system in monotherapy. Target organ damage is more frequent and blood pressure (BP) is more difficult to control despite more intensive therapy...
September 2016: Journal of Hypertension
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