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https://www.readbyqxmd.com/read/27922499/the-dystrophic-and-nondystrophic-myotonias
#1
Valeria A Sansone
PURPOSE OF REVIEW: This article describes clinical and electrical myotonia and provides an update on the classification, diagnosis, and management of myotonic disorders. RECENT FINDINGS: In the myotonic dystrophies, antisense oligonucleotides provide a general strategy to correct RNA gain of function and modulate the expression of CTG expanded repeats; they are currently being tested in a phase 1-2 randomized controlled trial in patients with adult-onset myotonic dystrophy type 1...
December 2016: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/27922190/clc-3-promotes-osteogenic-differentiation-in-mc3t3-e1-cell-after-dynamic-compression
#2
Dawei Wang, Hao Wang, Feng Gao, Kun Wang, Fusheng Dong
ClC-3 chloride channel has been proved to have a relationship with the expression of osteogenic markers during osteogenesis, persistent static compression can upregulate the expression of ClC-3 and regulate osteodifferentiation in osteoblasts. However, there was no study about the relationship between the expression of ClC-3 and osteodifferentiation after dynamic compression. In this study, we applied dynamic compression on MC3T3-E1 cells to detect the expression of ClC-3, runt-related transcription factor 2 (Runx2), bone morphogenic protein-2 (BMP-2), osteopontin (OPN), nuclear-associated antigen Ki67 (Ki67) and proliferating cell nuclear antigen (PCNA) in biopress system, then we investigated the expression of these genes after dynamic compression with Chlorotoxin (specific ClC-3 chloride channel inhibitor) added...
December 6, 2016: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/27921211/helix-o-modulates-voltage-dependency-of-clc-1
#3
Ju Yong Seong, Kotdaji Ha, Chansik Hong, Jongyun Myeong, Hyun-Ho Lim, Dongki Yang, Insuk So
The chloride channel (CLC) family of proteins consists of channels and transporters that share similarities in architecture and play essential roles in physiological functions. Among the CLC family, CLC-1 channels have the representative homodimeric double-barreled structure carrying two gating processes. One is protopore gating that acts on each pore independently by glutamate residue (Eext). The other is common gating that closes both pores simultaneously in association with large conformational changes across each subunit...
December 5, 2016: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/27921040/new-insights-into-the-molecular-mechanisms-targeting-tubular-channels-transporters-in-pkd-development
#4
REVIEW
Ming Wu, Shengqiang Yu
BACKGROUND: Autosomal dominant polycystic kidney disease (PKD) or autosomal recessive PKD is caused by a mutation in the PKD1, PKD2 or PKHD1 gene, which encodes polycystin-1, polycystin-2 or fibrocystin, respectively. Embryonic and postnatal mutation studies show that transport or channel function is dysregulated before the initiation of cystogenesis, suggesting that the abnormality of transport or channel function plays a critical role in the pathology of PKD. SUMMARY: Polycystin-2 by itself is a calcium-permeable cation channel, and its channel function can be regulated by polycystin-1 or fibrocystin...
October 2016: Kidney Diseases
https://www.readbyqxmd.com/read/27919319/chemogenetic-inhibition-of-the-medial-prefrontal-cortex-reverses-the-effects-of-rem-sleep-loss-on-sucrose-consumption
#5
Kristopher McEown, Yohko Takata, Yoan Cherasse, Nanae Nagata, Kosuke Aritake, Michael Lazarus
Rapid eye movement (REM) sleep loss is associated with increased consumption of weight-promoting foods. The prefrontal cortex (PFC) is thought to mediate reward anticipation. However, the precise role of the PFC in mediating reward responses to highly palatable foods (HPF) after REM sleep deprivation is unclear. We selectively reduced REM sleep in mice over a 25-48 hr period and chemogenetically inhibited the medial PFC (mPFC) by using an altered glutamate-gated and ivermectin-gated chloride channel that facilitated neuronal inhibition through hyperpolarizing infected neurons...
December 6, 2016: ELife
https://www.readbyqxmd.com/read/27917283/protective-effects-of-nimodipine-and-lithium-against-aluminum-induced-cell-death-and-oxidative-stress-in-pc12-cells
#6
Jamileh Saberzadeh, Mehdi Omrani, Mohammad Ali Takhshid
OBJECTIVES: The role of aluminum (Al) in the pathogenesis of neurodegenerative diseases has been implicated in several studies. However, the exact mechanisms of cytotoxic effects of Al have not been elucidated yet. The aim of this study was to investigate the effect of L-type calcium channel antagonist, nimodipine (NM), and lithium chloride (LiCl) on Al-induced toxicity in PC12 cells. MATERIALS AND METHODS: PC12 cells were treated with Al-maltolate (Almal) in the presence and absence of different concentrations of NM (50-150 μm) and/or LiCl (0...
November 2016: Iranian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/27913277/specific-stabilization-of-cftr-by-phosphatidylserine
#7
Ellen Hildebrandt, Netaly Khazanov, John C Kappes, Qun Dai, Hanoch Senderowitz, Ina L Urbatsch
The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR, ABCC7) is a plasma membrane chloride ion channel in the ABC transporter superfamily. CFTR is a key target for cystic fibrosis drug development, and its structural elucidation would advance those efforts. However, the limited in vivo and in vitro stability of the protein, particularly its nucleotide binding domains, has made structural studies challenging. Here we demonstrate that phosphatidylserine uniquely stimulates and thermally stabilizes the ATP hydrolysis function of purified human CFTR...
November 29, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27901358/mdck-cells-are-capable-of-water-secretion-and-reabsorption-in-response-to-changes-in-the-ionic-environment
#8
Janne P Capra, Sinikka M Eskelinen
A prerequisite for tissue electrolyte homeostasis is highly regulated ion and water transport through kidney or intestinal epithelia. In the present work, we monitored changes in the cell and luminal volumes of type II Madin-Darby canine kidney (MDCK) cells grown in a 3D environment in response to drugs, or to changes in the composition of the basal extracellular fluid. Using fluorescent markers and high-resolution spinning disc confocal microscopy, we could show that lack of sodium and potassium ions in the basal fluid (tetramethylammonium chloride (TMACl) buffer) induces a rapid increase in the cell and luminal volumes...
September 1, 2016: Canadian Journal of Physiology and Pharmacology
https://www.readbyqxmd.com/read/27899419/progressive-cl-channel-defects-reveal-disrupted-skeletal-muscle-maturation-in-r6-2-huntington-s-mice
#9
Daniel R Miranda, Monica Wong, Shannon H Romer, Cynthia McKee, Gabriela Garza-Vasquez, Alyssa C Medina, Volker Bahn, Andrew D Steele, Robert J Talmadge, Andrew A Voss
Huntington's disease (HD) patients suffer from progressive and debilitating motor dysfunction. Previously, we discovered reduced skeletal muscle chloride channel (ClC-1) currents, inwardly rectifying potassium (Kir) channel currents, and membrane capacitance in R6/2 transgenic HD mice. The ClC-1 loss-of-function correlated with increased aberrant mRNA processing and decreased levels of full-length ClC-1 mRNA (Clcn1 gene). Physiologically, the resulting muscle hyperexcitability may help explain involuntary contractions of HD...
November 29, 2016: Journal of General Physiology
https://www.readbyqxmd.com/read/27895116/correctors-and-potentiators-rescue-function-of-the-truncated-w1282x-cftr-translation-product
#10
Peter M Haggie, Puay-Wah Phuan, Joseph-Anthony Tan, Haijin Xu, Radu G Avramescu, Doranda Perdomo, Lorna Zlock, Dennis W Nielson, Walter E Finkbeiner, Gergely L Lukacs, Alan S Verkman
W1282X is the fifth most common CFTR mutation that causes cystic fibrosis. Here, we investigated the utility of a small molecule corrector / potentiator strategy, as used for ΔF508-CFTR, to produce functional rescue of the truncated translation product of the W1282X mutation, CFTR1281, without the need for read-through. In transfected cell systems, certain potentiators and correctors including VX-809 and VX-770 increased CFTR1281 activity. To identify novel correctors and potentiators with potentially greater efficacy on CFTR1281, functional screens were done of ~30,000 synthetic small molecules and drugs/nutraceuticals in CFTR1281-transfected cells...
November 28, 2016: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27888566/functional-expression-of-g-protein-coupled-receptor-30-in-immature-rat-epididymal-epithelium
#11
Xiaonian Cao, Jiehong Huang, Geng Zhang, Wulin Zuo, Chongfeng Lan, Qing Sun, Dengliang Yang, Dongdong Gao, Christopher H K Cheng, Wen-Liang Zhou
The aim of this study is to investigate the functional role of G protein-coupled receptor 30 (GPR30) in the epididymis. We found that GPR30 is expressed in the epithelium of the immature rat epididymis and is involved in chloride secretion into the caudal epididymis lumen. The short-circuit current (Isc) experiments showed that in primary cultured caudal epididymis epithelium, activation of GPR30 by its specific agonist G1 induced a mono-phasic current increase, and G15, the specific antagonist of GPR30, could completely inhibit the current induced by G1...
November 26, 2016: Cell Biology International
https://www.readbyqxmd.com/read/27888479/use-of-major-ions-to-evaluate-the-hydrogeochemistry-of-groundwater-influenced-by-reclamation-and-seawater-intrusion-west-nile-delta-egypt
#12
Zenhom El-Said Salem, Osman M Osman
The aim of this research is to evaluate the groundwater geochemistry in western Nile Delta area as an example of an aquifer influenced by reclamation and seawater intrusion. To conduct this study, 63 groundwater samples and one surface water sample from El Nubaria Canal were collected. To estimate the origin of dissolved ions and the geochemical processes influencing this groundwater, integration between land use change, pedological, hydrogeological, hydrogeochemical, and statistical approaches was considered...
November 25, 2016: Environmental Science and Pollution Research International
https://www.readbyqxmd.com/read/27886375/modulation-of-chloride-currents-in-human-lung-epithelial-cells-exposed-to-exogenous-oxidative-stress
#13
Rita Canella, Marta Martini, Roberta Borriello, Carlotta Cavicchio, Ximena M Muresan, Mascia Benedusi, Franco Cervellati, Giuseppe Valacchi
Air pollution continues to be a major public health concern affecting 9 out of 10 individuals living in urban areas worldwide. Respiratory tract is the organ most exposed to gas pollution and ozone has been shown to be one of the most noxious pollutants to which living organisms are exposed. In the present work, we have investigated the effects of 0.1 ppm of ozone on chloride currents in human lung epithelial cells (A549 line) and whether this effect could be modulated by vitamin E pre-treatment. Whole-cell patch clamp technique was applied to not excitable cells in order to obtain information about chloride currents behavior, important for epithelial lung cells homeostasis...
November 25, 2016: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/27881775/a-biophysical-model-for-cytotoxic-cell-swelling
#14
Koen Dijkstra, Jeannette Hofmeijer, Stephan A van Gils, Michel J A M van Putten
: We present a dynamic biophysical model to explain neuronal swelling underlying cytotoxic edema in conditions of low energy supply, as observed in cerebral ischemia. Our model contains Hodgkin-Huxley-type ion currents, a recently discovered voltage-gated chloride flux through the ion exchanger SLC26A11, active KCC2-mediated chloride extrusion, and ATP-dependent pumps. The model predicts changes in ion gradients and cell swelling during ischemia of various severity or channel blockage with realistic timescales...
November 23, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/27876591/cystic-fibrosis-and-the-nervous-system
#15
REVIEW
Leah R Reznikov
Cystic Fibrosis (CF) is a life-shortening autosomal recessive disorder caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). CFTR is an anion channel that conducts bicarbonate and chloride across cell membranes. While defective anion transport across epithelial cells is accepted as the basic defect in CF, many of the features observed in people with CF and organs affected in CF are modulated by the nervous system. This is of interest because CFTR expression has been reported in both the peripheral and central nervous systems, and it is well known that the transport of anions, such as chloride, greatly modulates neuronal excitability...
November 19, 2016: Chest
https://www.readbyqxmd.com/read/27871896/gastroprotective-and-ulcer-healing-effects-of-hydroethanolic-extract-of-leaves-of-caryocar-coriaceum-mechanisms-involved-in-the-gastroprotective-activity
#16
Luis Jardelino de Lacerda Neto, Andreza Guedes Barbosa Ramos, Valterlucio Santos Sales, Severino Denicio Gonçalves de Souza, Antonia Thassya Lucas Dos Santos, Larissa Rolim de Oliveira, Marta Regina Kerntopf, Thais Rodrigues de Albuquerque, Henrique Douglas Melo Coutinho, Lucindo Jose Quintans-Júnior, Almir Gonçalves Wanderley, Irwin Rose Alencar de Menezes
This work aimed to determine the chemical fingerprint of hydroethanolic extract of leaves of Caryocar coriaceum (HELCC) and the gastroprotective activity. The chemical fingerprint of HELCC was analyzed by HPLC-DAD, to quantify total phenols and flavonoids were carried out by Folin-Ciocalteu reagent and aluminum chloride assay, while in vitro antioxidant activity was evaluated by the DPPH method. The methods used to determine pharmacological activity were: gastroprotective screening test in classical models of induced acute and chronic gastric lesions and physical barrier test...
November 18, 2016: Chemico-biological Interactions
https://www.readbyqxmd.com/read/27871064/benzopyrimido-pyrrolo-oxazine-dione-cftr-inhibitor-r-bpo-27-for-anti-secretory-therapy-of-diarrheas-caused-by-bacterial-enterotoxins
#17
Onur Cil, Puay-Wah Phuan, Anne Marie Gillespie, Sujin Lee, Lukmanee Tradtrantip, Jianyi Yin, Ming Tse, Nicholas C Zachos, Ruxian Lin, Mark Donowitz, Alan S Verkman
Secretory diarrheas caused by bacterial enterotoxins, including cholera and traveler's diarrhea, remain a major global health problem. Inappropriate activation of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel occurs in these diarrheas. We previously reported that the benzopyrimido-pyrrolo-oxazinedione (R)-BPO-27 inhibits CFTR chloride conductance with low-nanomolar potency. Here, we demonstrate using experimental mouse models and human enterocyte cultures the potential utility of (R)-BPO-27 for treatment of secretory diarrheas caused by cholera and Escherichia coli enterotoxins...
November 8, 2016: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/27866192/dietary-genistein-rescues-reduced-basal-chloride-secretion-in-diabetic-jejunum-via-sex-dependent-mechanisms
#18
Shawn Catmull, Faisal Masood, Sydney Schacht, Robert Dolan, Daniel Stegman, Lana Leung, Layla Al-Nakkash
BACKGROUND/AIMS: The goal of this study was to determine the effect of dietary genistein (naturally occurring phytoestrogen) on jejunal secretory function in a clinically relevant model of diabetes and obesity, the leptin-defIcient ob/ob mouse. METHODS: We measured transepithelial short circuit current (Isc), across freshly isolated segments of jejunum from 12-week old male and female ob/ob and lean C57Bl/6J mice fed a genistein diet (600 mg genistein/kg diet) for 4-weeks...
2016: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/27861612/clic1-inhibition-attenuates-vascular-inflammation-oxidative-stress-and-endothelial-injury
#19
Yingling Xu, Ji Zhu, Xiao Hu, Cui Wang, Dezhao Lu, Chenxue Gong, Jinhuan Yang, Lei Zong
Endothelial dysfunction, which includes endothelial oxidative damage and vascular inflammation, is a key initiating step in the pathogenesis of atherosclerosis (AS) and an independent risk factor for this disorder. Intracellular chloride channel 1 (CLIC1), a novel metamorphic protein, acts as a sensor of cell oxidation and is involved in inflammation. In this study, we hypothesize that CLIC1 plays an important role in AS. Apolipoprotein E-deficient mice were supplied with a normal diet or a high-fat and high-cholesterol diet for 8 weeks...
2016: PloS One
https://www.readbyqxmd.com/read/27860832/anoctamins-tmem16-proteins-chloride-channels-flirting-with-lipids-and-extracellular-vesicles
#20
Jarred M Whitlock, H Criss Hartzell
Anoctamin/TMEM16 proteins exhibit diverse functions in cells throughout the body and are implicated in several human diseases. Although the founding members ANO1 (TMEM16A) and ANO2 (TMEM16B) are Ca(2+)-activated Cl(-) channels, most ANO paralogs are Ca(2+)-dependent phospholipid scramblases that serve as channels facilitating the movement (scrambling) of phospholipids between leaflets of the membrane bilayer. Phospholipid scrambling significantly alters the physical properties of the membrane and its landscape and has vast downstream signaling consequences...
November 16, 2016: Annual Review of Physiology
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