• Title/Summary/Keyword: Sodium channel

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A case of pseudohypoaldosteronism type 1 with a mutation in the mineralocorticoid receptor gene

  • Lee, Se-Eun;Jung, Yun-Hye;Han, Kyoung-Hee;Lee, Hyun-Kyung;Kang, Hee-Gyung;Ha, Il-Soo;Choi, Yong;Cheong, Hae-Il
    • Clinical and Experimental Pediatrics
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    • v.54 no.2
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    • pp.90-93
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    • 2011
  • Pseudohypoaldosteronism type 1 (PHA1) is a rare form of mineralocorticoid resistance characterized in newborns by salt wasting with dehydration, hyperkalemia and failure to thrive. This disease is heterogeneous in etiology and includes autosomal dominant PHA1 owing to mutations of the NR3C2 gene encoding the mineralocorticoid receptor, autosomal recessive PHA1 due to mutations of the epithelial sodium channel (ENaC) gene, and secondary PHA1 associated with urinary tract diseases. Amongst these diseases, autosomal dominant PHA1 shows has manifestations restricted to renal tubules including a mild salt loss during infancy and that shows a gradual improvement with advancing age. Here, we report a neonatal case of PHA1 with a NR3C2 gene mutation (a heterozygous c.2146_2147insG in exon 5), in which the patient showed failure to thrive, hyponatremia, hyperkalemia, and elevated plasma renin and aldosterone levels. This is the first case of pseudohypoaldosteronism type 1 confirmed by genetic analysis in Korea.

Effect of carbamazepine on tetrodotoxin-resistant Na+ channels in trigeminal ganglion neurons innervating to the dura

  • Han, Jin-Eon;Cho, Jin-Hwa;Nakamura, Michiko;Lee, Maan-Gee;Jang, Il-Sung
    • The Korean Journal of Physiology and Pharmacology
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    • v.22 no.6
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    • pp.649-660
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    • 2018
  • Migraine is a neurological disorder characterized by recurrent and disabling severe headaches. Although several anticonvulsant drugs that block voltagedependent $Na^+$ channels are widely used for migraine, far less is known about the therapeutic actions of carbamazepine on migraine. In the present study, therefore, we characterized the effects of carbamazepine on tetrodotoxin-resistant (TTX-R) $Na^+$ channels in acutely isolated rat dural afferent neurons, which were identified by the fluorescent dye DiI. The TTX-R $Na^+$ currents were measured in medium-sized DiIpositive neurons using the whole-cell patch clamp technique in the voltage-clamp mode. While carbamazepine had little effect on the peak amplitude of transient $Na^+$ currents, it strongly inhibited steady-state currents of transient as well as persistent $Na^+$ currents in a concentration-dependent manner. Carbamazepine had only minor effects on the voltage-activation relationship, the voltage-inactivation relationship, and the use-dependent inhibition of TTX-R $Na^+$ channels. However, carbamazepine changed the inactivation kinetics of TTX-R $Na^+$ channels, significantly accelerating the development of inactivation and delaying the recovery from inactivation. In the current-clamp mode, carbamazepine decreased the number of action potentials without changing the action potential threshold. Given that the sensitization of dural afferent neurons by inflammatory mediators triggers acute migraine headaches and that inflammatory mediators potentiate TTX-R $Na^+$ currents, the present results suggest that carbamazepine may be useful for the treatment of migraine headaches.

Numerical Analysis on Heat Transfer Characteristics of a Heat Pipe Type Solar Thermal Receiver Depending on End Wall Angle Variation (고온 히트파이프식 태양열 흡수기의 후벽 각도 변화에 따른 열전달 특성의 수치해석)

  • Park, Young-Hark;Jung, Eui-Guk;Boo, Joon-Hong;Kang, Yong-Heack
    • 한국태양에너지학회:학술대회논문집
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    • 2008.11a
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    • pp.226-231
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    • 2008
  • The heat transfer characteristics of a solar high-temperature receiver with heat pipes was investigated by numerical simulation. The concentration ratio of the solar receiver ranges 1000 and the concentrated heat is required to be transported to a certain distance for specific applications. This study deals with internal geometry variation of a solar receiver incorporating high-temperature heat pipe. The isothermal characteristics in the receiver section is of major concern. The diameter of the solar thermal receiver was 120 mm and the length was 400 mm. The angle of receiver end wall was varied between $0^{\circ}$ and $45^{\circ}$. The wall thickness of the heat pipe channel was 4mm and 48 axial channels of the same dimensions were attached to the outer wall of the receiver with even spacing in the circumferential direction. The channels are changed to high-temperature sodium heat pipes. Commercial softwares were employed to deal with the radiative heat transfer inside the receiver cavity and the conduction heat transfer along the channels. The numerical results are compared and analyzed from the view Point of high-temperature solar receiver.

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A Case of Severe Aconitine Intoxication with Ventricular Tachycardia, Successfully Treated by a Combination of Two Anti-arrhythmic Drugs (두 가지 항부정맥 약제를 병용 투여하여 성공적으로 치료한 심실빈맥이 동반된 부자중독 1례 보고)

  • Ryoo, Seung-Mok;Sohn, Chang-Hwan;Oh, Bum-Jin;Kim, Won;Lim, Kyoung-Soo
    • Journal of The Korean Society of Clinical Toxicology
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    • v.9 no.2
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    • pp.105-108
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    • 2011
  • Aconitine is an anti-inflammatory agent with therapeutic uses in oriental medicine as an analgesic and for treatment of stroke. Because of its sodium channel effect, aconitine can promote undesirable, wide complex tachyarrhythmia. If tachycardia develops during use of aconitine, class Ia and class III anti arrhythmic drugs can be utilized for treatment. However there are no single anti-arrhythmia agents which are uniformly effective. We report a case, characterized by wide complex tachyarrhythmia and severe hypotension, which was successfully treated by simultaneous injections of amiodarone and lidocaine. A 59-year-old woman exhibiting clinical signs of drowsiness as a result of ingesting 6 g of aconitine, was admitted to the emergency department. Initially, wide complex tachyarrhythmia (ventricular tachycardia and pulse rate of 180 beats/min) and severe hypotension (blood pressure of 53/26 mmHg) was observed. After simultaneous injection of amiodarone and lidocaine, the patient's rhythm pattern changed to an accelerated junctional rhythm with ventricular premature complex. Two hours later, the patient's heart pattern became a sinus rhythm. As demonstrated by this case, simultaneous injections of amiodarone and lidocaine can be useful in treating ventricular arrhythmia induced by aconitine.

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Design of A scale-down experimental model for SFR reactor vault cooling system performance analyses

  • Kim, Koung Moon;Hwang, Ji-Hwan;Wongwises, Somchai;Jerng, Dong-Wook;Ahn, Ho Seon
    • Nuclear Engineering and Technology
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    • v.52 no.8
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    • pp.1611-1625
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    • 2020
  • We propose a scaled-down experimental model of vertical air-natural convection channels by applying the modified Ishii-Kataoka scaling method with the assistance of numerical analyses to the Reactor Vault Cooling System (RVCS) of the Proto-type Gen-IV Sodium-cooled fast reactor (PGSFR) being developed in Korea. Two major non-dimensional numbers (modified Richardson and Friction number) from the momentum equation and Stanton number from the energy balance equation were identified to design the scaled-down experimental model to assimilate thermal-hydraulic behaviors of the natural convective air-cooling channel of RVCS. The ratios of the design parameters in the PGSFR RVCS between the prototype and the scaled-down model were determined by setting Richardson and Stanton number to be unity. The friction number which cannot be determined by the Ishii-Kataoka method was estimated by numerical analyses using the MARS-KS system code. The numerical analyses showed that the friction number with the form loss coefficient of 2.0 in the scale-down model would result in an acceptable prediction of the thermal-hydraulic behavior in RVCS. We also performed experimental benchmarking using the scaled-down model with the MARS-KS simulations to verify the appropriateness of the scale-down model, which demonstrated that the temperature rises and the average air flow velocity measured in the scale-down model.

CaCO3 Biomineralization in Microfluidic Crystallizer (미세유체 결정화기를 이용한 탄산칼슘 Biomineralization)

  • Seo, Seung Woo;Ko, Kwan Young;Lee, Chang Soo;Kim, In Ho
    • Korean Chemical Engineering Research
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    • v.51 no.1
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    • pp.151-156
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    • 2013
  • Crystallization of $CaCO_3$ is practiced on a polymethylsiloxane (PDMS) - based microfluidic system. Liquid- liquid reaction was investigated by mixing calcium chloride ($CaCl_2$) and sodium carbonate ($Na_2CO_3$) solution to crystallize $CaCO_3$. Aspartic acid (Asp) was added to investigate the morphology change such as vaterite and calcite. Suitable ratio of $Na_2CO_3$ and $CaCl_2$ was searched for initial seed formation. Christmas tree model was used as microfluidic device to form concentration gradient of $Na_2CO_3$ and $CaCl_2$. After observing microfluidic channel by using optical microscope, we found that seeds of $CaCO_3$ were formed under the condition that the ratio of $Na_2CO_3$ and $CaCl_2$ was 2:1. Morphology of crystals were also observed as $CaCO_3$ crystals grow. When Asp was added, vaterite crystal was more frequently found in two morphologies (vaterite and calcite) and seed formation and crystal growth were inhibited.

Reactive Oxygen Species and Nitrogen Species Differentially Regulate Neuronal Excitability in Rat Spinal Substantia Gelatinosa Neurons

  • Lee, Hae In;Park, A-Reum;Chun, Sang Woo
    • International Journal of Oral Biology
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    • v.39 no.4
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    • pp.229-236
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    • 2014
  • Reactive oxygen species (ROS) and nitrogen species (RNS) are implicated in cellular signaling processes and as a cause of oxidative stress. Recent studies indicate that ROS and RNS are important signaling molecules involved in nociceptive transmission. Xanthine oxidase (XO) system is a well-known system for superoxide anions ($O{_2}^{{\cdot}_-}$) generation, and sodium nitroprusside (SNP) is a representative nitric oxide (NO) donor. Patch clamp recording in spinal slices was used to investigate the role of $O{_2}^{{\cdot}_-}$ and NO on substantia gelatinosa (SG) neuronal excitability. Application of xanthine and xanthine oxidase (X/XO) compound induced membrane depolarization. Low concentration SNP ($10{\mu}M$) induced depolarization of the membrane, whereas high concentration SNP (1 mM) evoked membrane hyperpolarization. These responses were significantly decreased by pretreatment with phenyl N-tert-butylnitrone (PBN; nonspecific ROS and RNS scavenger). Addition of thapsigargin to an external calcium free solution for blocking synaptic transmission, led to significantly decreased X/XO-induced responses. Additionally, X/XO and SNP-induced responses were unchanged in the presence of intracellular applied PBN, indicative of the involvement of presynaptic action. Inclusion of GDP-${\beta}$-S or suramin (G protein inhibitors) in the patch pipette decreased SNP-induced responses, whereas it failed to decrease X/XO-induced responses. Pretreatment with n-ethylmaleimide (NEM; thiol-alkylating agent) decreased the effects of SNP, suggesting that these responses were mediated by direct oxidation of channel protein, whereas X/XO-induced responses were unchanged. These data suggested that ROS and RNS play distinct roles in the regulation of the membrane excitability of SG neurons related to the pain transmission.

Effects of Azumolene on Ryanodine Binging to Sarcoplasmic Reticulum of Normal and Malignant Hyperthermia Sucseptible Swine Skeletal Muscles

  • Kim, Do-Han;Lee, Young-Sup
    • Animal cells and systems
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    • v.1 no.1
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    • pp.77-80
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    • 1997
  • DOantrolene is a primary specific therapeutic drug for prevention and treatment of malignant hyperthermia symptoms. The mechanisms underlying the therapeutic effects of the drug are not well understood. The present study aimed at the characterization of the effects of azumolene, a water soluble dantrolene analogue, on ryanodine binding to sarcoplasmic reticulum (SR) from normal and malign::lnt hyperthermia susceptible (MHS) swine muscles. Characteristics of $[^3H]ryanodine$ binding were clearly different between the two types of SR. Kinetic analysis of eH]ryanodine binding to SR in the presence of $2{\mu}M$ $Ca^{2+}$ showed that association constant $(K_{ryanodine}_7$ is significantly higher in MHS than normal muscle SR $(2.83 vs. 1.32{\times}10^7 M^{-1}$, whereas the maximal ryanodine binding capacity $(B_{max})$ is similar between the two types of SR. Addition of azumolene $(e.g. 400{\mu}M)$ did not significantly alter both $K_{ryanodine}$ and $B_{max}$ of $[^3H]$ryanodine binding in both types of SR, indicating that the azumolene effect was not on the ryanodine binding sites. Addition of caffeine activated $[^3H]$ ryanodine binding in both types of SR, and caffeine sensitivity was significantly higher in MHS muscle SR than normal muscle SR $(K_{caffeine}:3.24 vs. 0.82 {\times} 10^2 M^{-l}). Addition of azumolene $(e.g.400{\mu}M)$ decreased Kcaffeine without significant change in $B_{max}$ in both types of SR suggesting that azumolene competes with caffeine binding site(s). These results suggest that malignant hyperthermia symptoms are caused at least in part by greater sensitivity of the MHS muscle SR to the $Ca^{2+}$ release drug(s), and that azumolene can reverse the symptoms by reducing the drug affinity to $Ca^{2+}$ release channels.

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Proteome-based Identification of Proteins Regulated by Aldosterone in Rat Kidney Cortex (프로테오믹스 기술을 통한 쥐의 신장 피질에서 알도스테론(Aldosterone)에 의해 조절되는 단백질 동정)

  • Song, Mi-Na;Jeon, Hong-Bae;Choi, Hyo-Jung;Kwon, Tae-Hwan;Baek, Moon-Chang
    • YAKHAK HOEJI
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    • v.54 no.3
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    • pp.180-186
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    • 2010
  • Aldosterone, mineralocorticoid hormone, has important functions related to the regulation of blood pressure and balance of fluids and electrolytes in the distal region of the nephron. By genomic and non-genomic action of aldosterone, the physiological kidney functions are modulated. However, many of them except several kind of sodium channel have not been identified and analyzed yet. In this study, proteomic technologies with two-dimensional gel electrophoresis (2-DE) gel using aldosterone rat model were applied to analyze and identify the aldosterone dependently expressed proteins in rat kidney cortex. As a result, the established aldosterone rat model exhibited the normal physiological responses to aldosterone and modulated proteins were identified, which included 15 increased and 3 decreased proteins on 2-DE analysis. Among them, 11 proteins were identified as changed proteins by LC-MS/MS analysis. These proteins identified as aldosterone induced proteins were involved in several cellular pathways such as cytoskeleton remodeling, energy metabolism, amino acid metabolism, and chaperone process. In conclusion, our data could provide the insights into the new mechanism underlying regulation of kidney functions by aldosterone.

EFFECTS OF Porphyromonas endodontalis LIPOPOLYSACCHARIDE ON MEMBRANE PERMEABILITY OF FIBROBLAST (Porphyromonas endodontalis의 Lipopolysaccharide가 섬유아세포의 세포막 투과성에 미치는 영향)

  • Kim, Jae-Hee;Kim, Min-Kyum;Yoon, Soo-Han
    • Restorative Dentistry and Endodontics
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    • v.24 no.3
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    • pp.437-446
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    • 1999
  • Porphyromonas endodontalis(P. endodontalis) is one of the important causative bacteria of pulpal and periapical disease. P. endodontalis has lipopolysaccharide(LPS) and it plays a major role in stimulating the synthesis and release of cytokines from immune cells and prostaglandin $E_2$ from host cells. The purpose of this study is to prepare LPS from P. endodontalis and to evaluate the effect of LPS on membrane permeability of fibroblast. P. endodontalis ATCC 35406 was cultured in anaerobic condition, and LPS was extracted. LPS was confirmed by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Human periodontal ligament cell, colon fibroblast(CCD-18Co, KCLB 21459) and skin fibroblast(Detroit 551, KCLB 10110) were perfused with 0.01% P. endodontalis LPS solution, high concentration of $K^+$ solution and $Ca^{2+}$-free solution, $Ca^{2+}$ concentration ratio was measured by microfluorometry. 1. Intracellular $Ca^{2+}$ concentration was not changed in human periodontal fibroblast and skin fibroblast(Detroit 551) stimulated by P. endodontalis LPS. 2. Intracellular $Ca^{2+}$ concentration was increased in colon fibroblast(CCD-18Co) stimulated by P. endodontalis LPS. 3. Colon fibroblast(CCD-18Co) has voltage dependent $Ca^{2+}$ channel activated by high concentration of $K^+$ solution. 4. P. endodontalis LPS has no effect on the increase of intracellular $Ca^{2+}$ concentration during perfusion of $Ca^{2+}$-free solution.

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