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a cellular structure that is postulated to exist in order to mediate between a chemical agent that acts on nervous tissue and the physiological response

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=sense organ / 受信装置

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/02/26 15:54:58」(JST)

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Ryanodine receptor 2 (RYR2) is a protein found primarily in cardiac muscle. In humans, it is encoded by the RYR2 gene.^[1]^[2]^[3]

Function[edit]

The RYR2 protein functions as a component of a calcium channel that supplies ions to the cardiac muscle. The channel is composed of RYR2 tetramers and FK506-binding proteins found in a 1:4 stoichiometric ratio. Calcium channel function is affected by the specific type of FK506 isomer interacting with the RYR2 protein, due to binding differences and other factors.^[4] RYR2 channels control many cellular functions, including mitochondrial metabolism, gene expression and cell survival, in addition to their most studied role in cardiomyocyte contraction.^[5]

Clinical significance[edit]

Mutations in the RYR2 gene are associated with catecholaminergic polymorphic ventricular tachycardia, stress-induced polymorphic ventricular tachycardia, and arrhythmogenic right ventricular dysplasia.^[6]

Mice with genetically reduced Ryr2 exhibit a lower basal heart rate and fatal arrythmias.^[7]

Interactions[edit]

Ryanodine receptor 2 has been shown to interact with:

AKAP6,^[8]^[9]
PRKACA,^[8]
PRKACB,^[8]
PRKACG,^[8] and
SRI.^[10]

References[edit]

^ Otsu K, Willard HF, Khanna VK, Zorzato F, Green NM, MacLennan DH (September 1990). "Molecular cloning of cDNA encoding the Ca2+ release channel (ryanodine receptor) of rabbit cardiac muscle sarcoplasmic reticulum". J Biol Chem 265 (23): 13472–83. PMID 2380170.
^ Otsu K, Fujii J, Periasamy M, Difilippantonio M, Uppender M, Ward DC, MacLennan DH (October 1993). "Chromosome mapping of five human cardiac and skeletal muscle sarcoplasmic reticulum protein genes". Genomics 17 (2): 507–9. doi:10.1006/geno.1993.1357. PMID 8406504.
^ Tiso N, Stephan DA, Nava A, Bagattin A, Devaney JM, Stanchi F, Larderet G, Brahmbhatt B, Brown K, Bauce B, Muriago M, Basso C, Thiene G, Danieli GA, Rampazzo A (February 2001). "Identification of mutations in the cardiac ryanodine receptor gene in families affected with arrhythmogenic right ventricular cardiomyopathy type 2 (ARVD2)". Hum Mol Genet 10 (3): 189–94. doi:10.1093/hmg/10.3.189. PMID 11159936.
^ Guo T, Cornea RL, Huke S, Camors E, Yang Y, Picht E, Fruen BR, Bers DM (June 2010). "Kinetics of FKBP12.6 binding to ryanodine receptors in permeabilized cardiac myocytes and effects on Ca sparks". Circ. Res. 106 (11): 1743–52. doi:10.1161/CIRCRESAHA.110.219816. PMC 2895429. PMID 20431056.
^ Bround, Michael; Michael Rich Wambolt, Dan S. Luciani, Jerzy E. Kulpa, Brian Rodrigues, Roger W. Brownsey, Michael F. Allard, James D. Johnson (15 May 2013). "Cardiomyocyte ATP production, metabolic flexibility, and survival require calcium flux through cardiac ryanodine receptors in vivo". Journal of Biological Chemistry 288 (26): 18975–86. doi:10.1074/jbc.M112.427062. PMID 23678000. Cite uses deprecated parameters (help)
^ "Entrez Gene: RYR2 ryanodine receptor 2 (cardiac)".
^ Bround MJ, Asghari P, Wambolt RB, Bohunek L, Smits C, Philit M, Kieffer TJ, Lakatta EG, Boheler KR, Moore ED, Allard MF, Johnson JD (September 2012). "Cardiac ryanodine receptors control heart rate and rhythmicity in adult mice". Cardiovasc. Res. 96 (3): 372–80. doi:10.1093/cvr/cvs260. PMID 22869620.
^ ^a ^b ^c ^d Marx SO, Reiken S, Hisamatsu Y, Jayaraman T, Burkhoff D, Rosemblit N, Marks AR (May 2000). "PKA phosphorylation dissociates FKBP12.6 from the calcium release channel (ryanodine receptor): defective regulation in failing hearts". Cell 101 (4): 365–76. doi:10.1016/S0092-8674(00)80847-8. PMID 10830164.
^ Marx SO, Reiken S, Hisamatsu Y, Gaburjakova M, Gaburjakova J, Yang YM, Rosemblit N, Marks AR (May 2001). "Phosphorylation-dependent regulation of ryanodine receptors: a novel role for leucine/isoleucine zippers". J. Cell Biol. 153 (4): 699–708. doi:10.1083/jcb.153.4.699. PMC 2192391. PMID 11352932.
^ Meyers MB, Pickel VM, Sheu SS, Sharma VK, Scotto KW, Fishman GI (November 1995). "Association of sorcin with the cardiac ryanodine receptor". J. Biol. Chem. 270 (44): 26411–8. doi:10.1074/jbc.270.44.26411. PMID 7592856.

External links[edit]

GeneReviews/NCBI/NIH/UW entry on Catecholaminergic Polymorphic Ventricular Tachycardia
GeneReviews/NCBI/NIH/UW entry on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy, Autosomal Dominant
OMIM entries on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy, Autosomal Dominant
RYR2 protein, human at the US National Library of Medicine Medical Subject Headings (MeSH)

This article on a gene on chromosome 1 is a stub. You can help Wikipedia by expanding it.

UpToDate Contents

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1. カテコラミン誘発多形性心室頻拍および他のQT間隔正常の多形性心室頻拍 catecholaminergic polymorphic ventricular tachycardia and other polymorphic ventricular tachycardias with a normal qt interval
2. 不整脈原性右室心筋症：解剖学、組織学、病因、および遺伝学 arrhythmogenic right ventricular cardiomyopathy anatomy histology pathogenesis and genetics
3. 小児におけるQRS幅の広い頻脈の原因 causes of wide qrs complex tachycardia in children
4. 溺死（水没事故） drowning submersion injuries
5. 明らかな器質的心疾患のない患者における突然の心停止 sudden cardiac arrest in the absence of apparent structural heart disease

English Journal

Magnolol and honokiol regulate the calcium-activated potassium channels signaling pathway in Enterotoxigenic Escherichia coli-induced diarrhea mice.

Deng Y1, Han X2, Tang S2, Xiao W3, Tan Z4, Zhou C2, Wang M2, Kang J2.
European journal of pharmacology.Eur J Pharmacol.2015 May 15;755:66-73. doi: 10.1016/j.ejphar.2015.03.002. Epub 2015 Mar 11.
To explore the regulatory mechanisms of magnolol and honokiol on calcium-activated potassium channels signaling pathway in Enterotoxigenic Escherichia coli (ETEC)-induced diarrhea mice, the concentrations of serum chloride ion (Cl(-)), sodium ion (Na(+)), potassium ion (K(+)) and calcium ion (Ca(2+)
PMID 25771451

Regionally diverse mitochondrial calcium signaling regulates spontaneous pacing in developing cardiomyocytes.

Zhang XH1, Wei H1, Šarić T2, Hescheler J2, Cleemann L1, Morad M3.
Cell calcium.Cell Calcium.2015 May;57(5-6):321-36. doi: 10.1016/j.ceca.2015.02.003. Epub 2015 Feb 18.
The quintessential property of developing cardiomyocytes is their ability to beat spontaneously. The mechanisms underlying spontaneous beating in developing cardiomyocytes are thought to resemble those of adult heart, but have not been directly tested. Contributions of sarcoplasmic and mitochondrial
PMID 25746147

Calcium release channel RyR2 regulates insulin release and glucose homeostasis.

Santulli G, Pagano G, Sardu C, Xie W, Reiken S, D'Ascia SL, Cannone M, Marziliano N, Trimarco B, Guise TA, Lacampagne A, Marks AR.
The Journal of clinical investigation.J Clin Invest.2015 May 1;125(5):1968-78. doi: 10.1172/JCI79273. Epub 2015 Apr 6.
The type 2 ryanodine receptor (RyR2) is a Ca2+ release channel on the endoplasmic reticulum (ER) of several types of cells, including cardiomyocytes and pancreatic β cells. In cardiomyocytes, RyR2-dependent Ca2+ release is critical for excitation-contraction coupling; however, a functional role for
PMID 25844899

Japanese Journal

細胞内カルシウム制御による心不全治療 (特集心不全に対する多面的治療戦略 : 基礎から臨床へ)

循環器内科 = Cardioangiology 78(2), 149-153, 2015-08
NAID 40020564536

β受容体制御による心不全治療 (特集心不全に対する多面的治療戦略 : 基礎から臨床へ)

循環器内科 = Cardioangiology 78(2), 112-118, 2015-08
NAID 40020564485

カチオン・カルシウムチャネルの農薬ターゲットとしての実際と可能性 (生命と農薬科学 : ターゲットを知る)

日本農薬学会誌 = Japanese journal of pesticide science 40(1), 68-74, 2015-02
NAID 40020386302

「リアノジン受容体2型」

Ryanodine receptor 2 (cardiac)
Identifiers
Symbols	RYR2; ARVC2; ARVD2; RYR-2; RyR; VTSIP
External IDs	OMIM: 180902 MGI: 99685 HomoloGene: 37423 IUPHAR: RyR2 ChEMBL: 4403 GeneCards: RYR2 Gene
Gene Ontology
Molecular function	• intracellular ligand-gated calcium channel activity; • ryanodine-sensitive calcium-release channel activity; • calcium channel activity; • calcium ion binding; • protein binding; • calmodulin binding; • calcium-release channel activity; • protein kinase A catalytic subunit binding; • protein kinase A regulatory subunit binding; • identical protein binding; • protein self-association; • suramin binding; • ion channel binding; • calcium-induced calcium release activity;
Cellular component	• plasma membrane; • junctional sarcoplasmic reticulum membrane; • membrane; • sarcoplasmic reticulum; • Z disc; • sarcoplasmic reticulum membrane; • calcium channel complex; • protein complex;
Biological process	• response to hypoxia; • regulation of heart rate; • embryonic heart tube morphogenesis; • left ventricular cardiac muscle tissue morphogenesis; • cardiac muscle hypertrophy; • detection of calcium ion; • calcium ion transport; • cellular calcium ion homeostasis; • intrinsic apoptotic signaling pathway in response to osmotic stress; • positive regulation of heart rate; • regulation of cardiac muscle contraction by regulation of the release of sequestered calcium ion; • release of sequestered calcium ion into cytosol by sarcoplasmic reticulum; • calcium-mediated signaling; • BMP signaling pathway; • response to caffeine; • ion transmembrane transport; • calcium-mediated signaling using intracellular calcium source; • release of sequestered calcium ion into cytosol; • positive regulation of sequestering of calcium ion; • cytosolic calcium ion homeostasis; • response to redox state; • transmembrane transport; • regulation of cardiac muscle contraction; • cardiac muscle contraction; • canonical Wnt receptor signaling pathway; • calcium ion transport into cytosol; • sarcoplasmic reticulum calcium ion transport; • cellular response to caffeine; • establishment of protein localization to endoplasmic reticulum; • regulation of ventricular cardiac muscle cell action potential; • Purkinje myocyte to ventricular cardiac muscle cell signaling; • cell communication by electrical coupling involved in cardiac conduction; • positive regulation of ryanodine-sensitive calcium-release channel activity by adrenergic receptor signaling pathway involved in positive regulation of cardiac muscle contraction; • positive regulation of calcium-transporting ATPase activity;
	Sources: Amigo / QuickGO
Orthologs
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