chloride channel

WordNet

1. direct the flow of; "channel information towards a broad audience" (同)canalize, canalise
2. a television station and its programs; "a satellite TV channel"; "surfing through the channels"; "they offer more than one hundred channels" (同)television channel, TV channel
3. a passage for water (or other fluids) to flow through; "the fields were crossed with irrigation channels"; "gutters carried off the rainwater into a series of channels under the street"
4. (often plural) a means of communication or access; "it must go through official channels"; "lines of communication were set up between the two firms" (同)communication channel, line
5. a path over which electrical signals can pass; "a channel is typically what you rent from a telephone company" (同)transmission channel
6. a deep and relatively narrow body of water (as in a river or a harbor or a strait linking two larger bodies) that allows the best passage for vessels; "the ship went aground in the channel"
7. any compound containing a chlorine atom
8. any salt of hydrochloric acid (containing the chloride ion)
9. official routes of communication; "you have to go through channels"

PrepTutorEJDIC

1. 〈C〉『水路』(川・湾・運河の船の通行ができる深い部分) / 〈U〉河床・川底 / 〈C〉『海峡』 / 〈C〉みぞ(groove),(道路の)水渠(すいきょ) / 《複数形で》(運搬・伝達の)正式の経路(手続き);(一般に)経路 / 〈C〉(テレビ・ラジオの)チャンネル / …‘に'水路を開く / …‘に'みぞを堀る / …'を'伝える,流す
2. 塩化物

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/08/29 18:54:20」(JST)

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Clc chloride channel
Identifiers
Symbol	Voltage_CLC
Pfam	PF00654
InterPro	IPR014743
SCOP	1kpl
SUPERFAMILY	1kpl
TCDB	1.A.11
OPM superfamily	10
OPM protein	1ots
CDD	cd00400
Available protein structures: Pfam structures PDB RCSB PDB; PDBe; PDBj PDBsum structure summary

Chloride channels are a superfamily of poorly understood ion channels consisting of approximately 13 members.

Chloride channels display a variety of important physiological and cellular roles that include regulation of pH, volume homeostasis, organic solute transport, cell migration, cell proliferation and differentiation. Based on sequence homology the chloride channels can be subdivided into a number of groups. The importance of one such group, the CLC family of chloride channels, can be seen from the diseases that develop when the channel does not function normally.

This family of ion channels contains 10 or 12 transmembrane helices. Each protein forms a single pore. It has been shown that some members of this family form homodimers. In terms of primary structure, they are unrelated to known cation channels or other types of anion channels. Three CLC subfamilies are found in animals. CLC-1 (P35523) is involved in setting and restoring the resting membrane potential of skeletal muscle, while other channels play important parts in solute concentration mechanisms in the kidney [3]. These proteins contain two CBS domains. Chloride channels are also important for maintaining safe ion concentrations within plant cells.

Pathology

Bartter's syndrome, which is associated with renal salt wasting and hypokalemic alkalosis, is due to the defective transport of chloride ions and associated ions in the thick ascending loop of Henle. CLCNKB has been implicated.

Another inherited disease that affects the kidney organs is Dent's Disease, characterised by low molecular weight proteinuria and hypercalciuria where mutations in CLCN5 are implicated.

Thomsen disease is associated with dominant mutations and Becker disease with recessive mutations in CLCN1.

Cystic fibrosis is caused by a mutation in the DF508 region of the CFTR gene, which prevents the proper folding of the protein and subsequent degradation, resulting in decreased numbers of chloride channels in the body. This causes the buildup of mucus in the body and chronic infections.

Functions

Chloride channels are important for setting cell resting membrane potential and maintaining proper cell volume. These channels conduct Cl⁻ as well as other anions such as HCO₃⁻, I⁻, SCN⁻, and NO₃⁻. The structure of these channels are not like other known channels. Chloride channel subunits contain between 1 and 12 transmembrane segments. Some members of this family are activated by voltage, while others are activated by Ca²⁺, extracellular ligands, and pH among other modulators.

Commercial Applications

Some organic materials disrupt chloride channels in fleas, causing death. Selamectin is the active ingredient in Revolution, a topical insecticide and antihelminthic used on dogs and cats. Selamectin works by replacing glutamate which normally interacts with receptors that open chloride channels at muscle synapses found in parasites. Unlike glutamate, selamectin activates the chloride current without desensitization, thereby producing prolonged hyperpolarization and impaired muscle contraction.

Human genes

• CLCA1, CLCA2, CLCA3, CLCA4
• CLCN1, CLCN2, CLCN3, CLCN4, CLCN5, CLCN6, CLCN7, CLCNKA, CLCNKB
• CLIC1, CLIC2, CLIC3, CLIC4, CLIC5, CLIC6

See also

• Cystic fibrosis transmembrane conductance regulator
• Calcium-activated chloride channel

References

Further reading

• Schmidt-Rose T, Jentsch TJ (August 1997). "Reconstitution of functional voltage-gated chloride channels from complementary fragments of CLC-1". J. Biol. Chem. 272 (33): 20515–21. doi:10.1074/jbc.272.33.20515. PMID 9252364. 
• Zhang J, George AL Jr, Griggs RC, Fouad GT, Roberts J, Kwieciński H, Connolly AM, Ptácek LJ (October 1996). "Mutations in the human skeletal muscle chloride channel gene (CLCN1) associated with dominant and recessive myotonia congenita". Neurology 47 (4): 993–8. doi:10.1212/wnl.47.4.993. PMID 8857733. 
• Mindell JA, Maduke M (2001). "ClC chloride channels". Genome Biol. 2 (2): REVIEWS3003. doi:10.1186/gb-2001-2-2-reviews3003. PMC 138906. PMID 11182894. 
• Singh H (2010). "Two decades with dimorphic Chloride Intracellular Channels (CLICs)". Febs letters. 584 (10): 2112–21. doi:10.1016/j.febslet.2010.03.013. PMID 20226783. 

External links

• Chloride channels at the US National Library of Medicine Medical Subject Headings (MeSH)
• UMich Orientation of Proteins in Membranes families/superfamily-10 - Clc Chloride channels

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. デント病（X連鎖劣性腎結石症） dents disease x linked recessive nephrolithiasis
• 2. バーター症候群およびギテルマン症候群 bartter and gitelman syndromes
• 3. 嚢胞性線維症：実験的治療 cystic fibrosis investigational therapies
• 4. 嚢胞性線維症：遺伝学と病因 cystic fibrosis genetics and pathogenesis
• 5. 利尿剤とカルシウムバランス diuretics and calcium balance

English Journal

• Barium chloride impaired Kir2.1 inward rectification in its stably transfected HEK 293 cell lines.

• Shen N1, Zheng J2, Liu H3, Xu K4, Chen Q4, Chen Y5, Shen Y5, Jiang L2, Chen Y6.Author information 1Cardiovascular Division, Zhejiang Province People׳s Hospital, 158 Shangtang Road, Hangzhou 310014, PR China.2Cardiovascular Division, Jiaxing No. 2 Hospital, 1882 Central Circle Road South, Jiaxing 314001, PR China.3Chinese Herb Medicine Division, The Nurturing Station for the State Key Laboratory of Subtropical Silviculture, Zhejiang Agriculture and Forestry University, 88 North Circle Road, Lin'an 311300, PR China.4Safety Pharmacology Division, Olivepharmasolutions Ltd., 333 Changhong Road, Wukang 313200, PR China.5Pathophysiolog department, Medical School, Zhejiang University, Hangzhou 310058, PR China.6Chinese Herb Medicine Division, The Nurturing Station for the State Key Laboratory of Subtropical Silviculture, Zhejiang Agriculture and Forestry University, 88 North Circle Road, Lin'an 311300, PR China. Electronic address: ychen@zafu.edu.cn.AbstractKir2.1 channel is a typical inward rectified channel with little outward currents when the membrane depolarized. Barium blocks the inward Kir2.1 currents in a voltage-dependent manner. However, in this study we found that barium would impair the rectification and open Kir2.1 outward currents at a depolarized voltage, causing increment of outward current amplitudes by 43±7% (n=5, P<0.01) after 200s barium application. In the meanwhile, a higher barium concentration did block the outward currents by 17.5±4.3% (n=4, P<0.01) and temporarily twisted current upward tendency. The increment was likely barium specific since both calcium and Kir2.1 specific blocker, Chloroethylclonidine (CEC), did not enhance the current amplitudes. The rectification of Kir2.1 was not recovered by washing barium off, which suggested a non-competitive mechanism. Since the currents occurred at phase 1, 2 of cardiac action potential, it would likely shorten the action potential plateau and it would decrease QT duration in electrocardiography (ECG).
• European journal of pharmacology.Eur J Pharmacol.2014 May 5;730:164-70. doi: 10.1016/j.ejphar.2014.02.025. Epub 2014 Mar 12.
• Kir2.1 channel is a typical inward rectified channel with little outward currents when the membrane depolarized. Barium blocks the inward Kir2.1 currents in a voltage-dependent manner. However, in this study we found that barium would impair the rectification and open Kir2.1 outward currents at a de
• PMID 24631257

• Esophageal desalination is mediated by Na(+), H(+) exchanger-2 in the gulf toadfish (Opsanus beta).

• Esbaugh AJ1, Grosell M2.Author information 1University of Texas at Austin, Marine Science Institute, Austin, TX 78373, USA. Electronic address: a.esbaugh@austin.utexas.edu.2Rosenstiel School of Marine Science, University of Miami, Miami, FL 33149, USA.AbstractEsophageal desalination is a crucial step in the gastrointestinal water absorption pathway, as this pre-intestinal processing establishes the osmotic conditions necessary for water absorption. Previous work has shown that esophageal Na(+) absorption is amiloride sensitive; however, it is as yet unclear if Na(+), H(+) exchangers (NHE) or Na(+) channels (ENaC) are responsible. The purpose of the current study was therefore to investigate the roles that NHE isoforms may play in this process in a marine teleost, the gulf toadfish (Opsanus beta), as well as what role NHE isoforms may play in the downstream intestinal Na(+) transport. A combination of symmetrical current clamp and asymmetrical voltage clamp experiments showed the esophagus to contain both an ion absorptive current (Isc=0.83±0.68) and serosal side negative transepithelial potential (TEP=-4.9±0.6). (22)Na uptake (J(Na)m→s) was inhibited by 0.5mM EIPA, with no effect of 0.1mM amiloride, 1mM furosemide or 1mM thiazide. A Cl(-) free saline reduced J(Na)m→s by 40% while also reducing conductance and reversing TEP. These results suggest that both transcellular and paracellular components contribute to esophageal Na(+) transport, with transcellular transport mediated by NHE. The NHE1, NHE2 and NHE3 genes were amplified and tissue distribution analysis by real-time PCR showed high NHE2 expression levels in the esophagus and stomach. Little NHE3 expression was observed throughout the gastrointestinal tract, and NHE2 expression was absent from the intestine. Hypersalinity (60ppt) had no effect on the expression profile of NHE2, slc4a2, scl26a6, CAc or V-type ATPase (β-subunit), suggesting that esophageal desalination is less flexible in response to osmotic stress than the intestine.
• Comparative biochemistry and physiology. Part A, Molecular & integrative physiology.Comp Biochem Physiol A Mol Integr Physiol.2014 May;171:57-63. doi: 10.1016/j.cbpa.2014.02.012. Epub 2014 Feb 16.
• Esophageal desalination is a crucial step in the gastrointestinal water absorption pathway, as this pre-intestinal processing establishes the osmotic conditions necessary for water absorption. Previous work has shown that esophageal Na(+) absorption is amiloride sensitive; however, it is as yet uncl
• PMID 24548910

Japanese Journal

• A large cohort of myotonia congenita probands : novel mutations and a high-frequency mutation region in exons 4 and 5 of the CLCN1 gene

• Brugnoni Raffaella,Kapetis Dimos,Imbrici Paola [他]
• Journal of human genetics 58(9), 581-587, 2013-09
• NAID 40019791907

• 骨格筋型塩化物イオンチャネル遺伝子(CLCN1)の複合ヘテロ接合体変異で重症化したThomsen病

• 佐々木 良元,高橋 正紀,穀内 洋介 [他]
• 臨床神経学 53(4), 316-319, 2013-04
• NAID 40019663429

Related Links

• Chloride channel | Facebook

Chloride channel. 0 likes · 0 talking about this. ... Wikipedia Content from the Wikipedia article Chloride channel (contributors) licensed under CC-BY-SA Freebase Content from Freebase licensed under CC-BY

• CLCNKB - chloride channel, voltage-sensitive Kb - Genetics ...

The official name of this gene is “chloride channel, voltage-sensitive Kb.” CLCNKB is the gene's official symbol. The CLCNKB gene is also known by other names, listed below. Read more about gene names and symbols ...

Related Pictures

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★リンクテーブル★

リンク元	「駆虫薬」「クロライドチャンネル」「クロライドチャネル」「Cl- channel」「塩素イオンチャネル」
拡張検索	「voltage-dependent chloride channelopathy」「chloride channelopathy」
関連記事	「channel」「chloride」

「駆虫薬」

　　[★]

英

anthelmintic, anthelmintics

ラ

anthelmintica

関

http://en.wikipedia.org/wiki/Anthelmintic

antiplatyhelmintic agents	Antitrematodals (schistosomicides)	binds tubulin	benzimidazole (Triclabendazole#)
		acetylcholinesterase inhibitor	phosphonic acid (metrifonate)
		Other/unknown	quinoline (praziquantel#, oxamniquine#) · phenol (bithionol) · thiazole ([[niridazole]) · arylsulfonate (stibophen)
	anticestodals (taeniacides)	binds tubulin	benzimidazole (albendazole#)
		Other/unknown	salicylanilide (niclosamide)# · aminoacridine (quinacrine) · butyrophenone (desaspidin) · chlorophenol (dichlorophen)
antinematodal agents (macrofilaricides)		binds tubulin	benzimidazole (mebendazole#, thiabendazole, albendazole#, fenbendazole, ciclobendazole, flubendazole)
		chloride channel	macrolide (ivermectin#)
		NMDA	tetrahydropyrimidine (pyrantel#, pyrantel pamoate, oxantel)
		Other/unknown	piperazine (piperazine · diethylcarbamazine#) · thiazole (levamisole#) · quinolinium (pyrvinium) · benzylammonium (bephenium) · naphthalenesulfonate (suramin#)  · tribendimidine

「クロライドチャンネル」

　　[★]

英

chloride channel、Cl- channel

関

クロライドチャネル、塩素イオンチャネル、Clチャネル

「クロライドチャネル」

　　[★]

英

chloride channel, Cl^- channel, Cl channel

同

塩素イオンチャネル

「Cl- channel」

　　[★]

クロライドチャネル、Clチャネル

関

chloride channel、Cl channel

「塩素イオンチャネル」

　　[★]

英

chloride channel, クロライドチャネル

関

Cl

「voltage-dependent chloride channelopathy」

　　[★] 電位依存性塩素イオンチャネル病

「chloride channelopathy」

　　[★] 塩素イオンチャネル病

「channel」

　　[★]

• n.

• 川床、川底。水路、河道
• 海峡
• 水管、導管
• 溝
• 経路、ルート。道筋、方向

• チャネル、チャンネル、イオンチャネル

関

ion channel、ionic channel

　 　 　 　 　 　 　

「chloride」

　　[★] 塩素イオン