Ryr2

Summary

Gene Symbol: Ryr2
Description: ryanodine receptor 2, cardiac
Alias: 9330127I20Rik, RYR-2, ryanodine receptor 2, calcium release channel, cardiac muscle ryanodine receptor, cardiac muscle ryanodine receptor-calcium release channel, ryanodine receptor type 2, type 2 ryanodine receptor
Species: mouse

Top Publications

  1. ncbi Abnormal expression of genes associated with development and inflammation in the heart of mouse maternal phenylketonuria offspring
    R Matalon
    Department of Pediatrics, The University of Texas Medical Branch UTMB, Galveston, Texas 77555 0632, USA
    Int J Immunopathol Pharmacol 18:557-65. 2005
  2. pmc Leaky RyR2 trigger ventricular arrhythmias in Duchenne muscular dystrophy
    Jeremy Fauconnier
    Institut National de la Santé et de la Recherche Médical U637, Physiopathologie Cardiovasculaire, F 34295 Montpellier, France
    Proc Natl Acad Sci U S A 107:1559-64. 2010
  3. doi Na+-dependent SR Ca2+ overload induces arrhythmogenic events in mouse cardiomyocytes with a human CPVT mutation
    Simon Sedej
    Division of Cardiology, Medical University of Graz, Auenbruggerplatz 15, Graz A 8036, Austria
    Cardiovasc Res 87:50-9. 2010
  4. pmc The ryanodine receptor/calcium channel genes are widely and differentially expressed in murine brain and peripheral tissues
    G Giannini
    European Molecular Biology Laboratory, Heidelberg, Germany
    J Cell Biol 128:893-904. 1995
  5. pmc Requirement for the ryanodine receptor type 3 for efficient contraction in neonatal skeletal muscles
    F Bertocchini
    DIBIT, San Raffaele Scientific Institute, Milano, Italy
    EMBO J 16:6956-63. 1997
  6. pmc Embryonic lethality and abnormal cardiac myocytes in mice lacking ryanodine receptor type 2
    H Takeshima
    Department of Pharmacology, Faculty of Medicine, University of Tokyo, Hongo, Bunkyo ku, Tokyo, Japan
    EMBO J 17:3309-16. 1998
  7. ncbi Identification of mutations in the cardiac ryanodine receptor gene in families affected with arrhythmogenic right ventricular cardiomyopathy type 2 (ARVD2)
    N Tiso
    Department of Biology, University of Padova, 35121 Padova, Italy
    Hum Mol Genet 10:189-94. 2001
  8. pmc Molecular basis of Ca(2)+ activation of the mouse cardiac Ca(2)+ release channel (ryanodine receptor)
    P Li
    Cardiovascular Research Group, University of Calgary, Calgary, Alberta, Canada T2N 4N1
    J Gen Physiol 118:33-44. 2001
  9. pmc The ryanodine receptor modulates the spontaneous beating rate of cardiomyocytes during development
    Huang Tian Yang
    National Institute on Aging, Laboratory of Cardiovascular Science, National Institutes of Health, Baltimore, MD 21224, USA
    Proc Natl Acad Sci U S A 99:9225-30. 2002
  10. ncbi FKBP12.6 deficiency and defective calcium release channel (ryanodine receptor) function linked to exercise-induced sudden cardiac death
    Xander H T Wehrens
    Department of Physiology and Cellular Biophysics, Center for Molecular Cardiology, Columbia University College of Physicians and Surgeons, New York, NY 10032, USA
    Cell 113:829-40. 2003

Scientific Experts

Detail Information

Publications116 found, 100 shown here

  1. ncbi Abnormal expression of genes associated with development and inflammation in the heart of mouse maternal phenylketonuria offspring
    R Matalon
    Department of Pediatrics, The University of Texas Medical Branch UTMB, Galveston, Texas 77555 0632, USA
    Int J Immunopathol Pharmacol 18:557-65. 2005
    ..One step real time RT-PCR showed a reduction in cardiac troponin I, troponin T2 and ryanodine receptor 2. Determination of troponin I and T protein levels showed reduced levels of these proteins...
  2. pmc Leaky RyR2 trigger ventricular arrhythmias in Duchenne muscular dystrophy
    Jeremy Fauconnier
    Institut National de la Santé et de la Recherche Médical U637, Physiopathologie Cardiovasculaire, F 34295 Montpellier, France
    Proc Natl Acad Sci U S A 107:1559-64. 2010
    ..and functional remodeling of the cardiac sarcoplasmic reticulum (SR) Ca(2+) release channel/ryanodine receptor (RyR2) occurs in the mdx mouse model of DMD. RyR2 from mdx hearts were S-nitrosylated and depleted of calstabin2 (FKBP12...
  3. doi Na+-dependent SR Ca2+ overload induces arrhythmogenic events in mouse cardiomyocytes with a human CPVT mutation
    Simon Sedej
    Division of Cardiology, Medical University of Graz, Auenbruggerplatz 15, Graz A 8036, Austria
    Cardiovasc Res 87:50-9. 2010
    Mutations in the cardiac ryanodine receptor Ca(2+) release channel, RyR2, underlie catecholaminergic polymorphic ventricular tachycardia (CPVT), an inherited life-threatening arrhythmia...
  4. pmc The ryanodine receptor/calcium channel genes are widely and differentially expressed in murine brain and peripheral tissues
    G Giannini
    European Molecular Biology Laboratory, Heidelberg, Germany
    J Cell Biol 128:893-904. 1995
    ..RyR mRNAs were also detected in in vitro-cultured cell lines. RyR1, RyR2, and RyR3 mRNA were detected in the cerebrum and in the cerebellum...
  5. pmc Requirement for the ryanodine receptor type 3 for efficient contraction in neonatal skeletal muscles
    F Bertocchini
    DIBIT, San Raffaele Scientific Institute, Milano, Italy
    EMBO J 16:6956-63. 1997
    ..The disproportion between the low amount of RyR3 and the large impact of the RyR3 knockout suggests that this isoform contributes to the amplification of Ca2+ released by the existing population of ryanodine receptors (RyR1)...
  6. pmc Embryonic lethality and abnormal cardiac myocytes in mice lacking ryanodine receptor type 2
    H Takeshima
    Department of Pharmacology, Faculty of Medicine, University of Tokyo, Hongo, Bunkyo ku, Tokyo, Japan
    EMBO J 17:3309-16. 1998
    The ryanodine receptor type 2 (RyR-2) functions as a Ca2+-induced Ca2+ release (CICR) channel on intracellular Ca2+ stores and is distributed in most excitable cells with the exception of skeletal muscle cells...
  7. ncbi Identification of mutations in the cardiac ryanodine receptor gene in families affected with arrhythmogenic right ventricular cardiomyopathy type 2 (ARVD2)
    N Tiso
    Department of Biology, University of Padova, 35121 Padova, Italy
    Hum Mol Genet 10:189-94. 2001
    ..genes (actinin 2 and nidogen), elucidation of the genomic structure of the cardiac ryanodine receptor gene (RYR2) and identification of RYR2 mutations in four independent families...
  8. pmc Molecular basis of Ca(2)+ activation of the mouse cardiac Ca(2)+ release channel (ryanodine receptor)
    P Li
    Cardiovascular Research Group, University of Calgary, Calgary, Alberta, Canada T2N 4N1
    J Gen Physiol 118:33-44. 2001
    Activation of the cardiac ryanodine receptor (RyR2) by Ca(2)+ is an essential step in excitation-contraction coupling in heart muscle. However, little is known about the molecular basis of activation of RyR2 by Ca(2)+...
  9. pmc The ryanodine receptor modulates the spontaneous beating rate of cardiomyocytes during development
    Huang Tian Yang
    National Institute on Aging, Laboratory of Cardiovascular Science, National Institutes of Health, Baltimore, MD 21224, USA
    Proc Natl Acad Sci U S A 99:9225-30. 2002
    ..depressed in ES cell-derived cardiomyocytes with a functional knockout (KO) of the cardiac ryanodine receptor (RyR2). KO myocytes show a slowing of the rate of spontaneous diastolic depolarization and an absence of calcium sparks...
  10. ncbi FKBP12.6 deficiency and defective calcium release channel (ryanodine receptor) function linked to exercise-induced sudden cardiac death
    Xander H T Wehrens
    Department of Physiology and Cellular Biophysics, Center for Molecular Cardiology, Columbia University College of Physicians and Surgeons, New York, NY 10032, USA
    Cell 113:829-40. 2003
    ..In cardiac muscle, the ryanodine receptor (RyR2) on the sarcoplasmic reticulum releases the calcium required for muscle contraction...
  11. ncbi Ryanodine receptor mutations associated with stress-induced ventricular tachycardia mediate increased calcium release in stimulated cardiomyocytes
    Christopher H George
    Wales Heart Research Institute, University of Wales College of Medicine, Heath Park, Cardiff, UK
    Circ Res 93:531-40. 2003
    Ca2+ release from the sarcoplasmic reticulum mediated by the cardiac ryanodine receptor (RyR2) is a fundamental event in cardiac muscle contraction...
  12. ncbi Residue Gln4863 within a predicted transmembrane sequence of the Ca2+ release channel (ryanodine receptor) is critical for ryanodine interaction
    Ruiwu Wang
    Cardiovascular Research Group, Department of Physiology and Biophysics, University of Calgary, Calgary, Alberta T2N 4N1, Canada
    J Biol Chem 278:51557-65. 2003
    ..Each amino acid residue within the TM10 sequence, 4844IIFDITFFFFVIVILLAIIQGLII4867, of the mouse RyR2 was mutated to either alanine or glycine...
  13. ncbi FKBP12.6 and cADPR regulation of Ca2+ release in smooth muscle cells
    Yong Xiao Wang
    Center for Cardiovascular Sciences, Albany Medical College, Albany, New York 12208, USA
    Am J Physiol Cell Physiol 286:C538-46. 2004
    ..Here we show that FK506 binding protein of 12.6 kDa (FKBP12.6) associates with and regulates type 2 RyRs (RyR2) in tracheal smooth muscle. FKBP12...
  14. ncbi Ryanodine receptors in human pancreatic beta cells: localization and effects on insulin secretion
    James D Johnson
    Division of Metabolism, Department of Internal Medicine, Washington University School of Medicine, Box 8126, 8831 Wohl Clinic, 660 S Euclid, St Louis, MO 63110, USA
    FASEB J 18:878-80. 2004
    ....
  15. ncbi RyR2 and calpain-10 delineate a novel apoptosis pathway in pancreatic islets
    James D Johnson
    Department of Internal Medicine, Washington University, St Louis, Missouri 63110, USA
    J Biol Chem 279:24794-802. 2004
    Cells are programmed to die when critical signaling and metabolic pathways are disrupted. Inhibiting the type 2 ryanodine receptor (RyR2) in human and mouse pancreatic beta-cells markedly increased apoptosis...
  16. ncbi Protection from cardiac arrhythmia through ryanodine receptor-stabilizing protein calstabin2
    Xander H T Wehrens
    Department of Physiology and Cellular Biophysics, Columbia University College of Physicians and Surgeons, New York, NY 10032, USA
    Science 304:292-6. 2004
    ..6) from the ryanodine receptor-calcium release channel (RyR2) complex causes an intracellular Ca2+ leak that can trigger fatal cardiac arrhythmias...
  17. ncbi Bidirectional ventricular tachycardia and fibrillation elicited in a knock-in mouse model carrier of a mutation in the cardiac ryanodine receptor
    Marina Cerrone
    Molecular Cardiology, IRCCS Fondazione S Maugeri, Pavia, Italy
    Circ Res 96:e77-82. 2005
    ..The autosomal dominant form of CPVT is caused by mutations in the RyR2 gene encoding the cardiac isoform of the ryanodine receptor...
  18. pmc Localization of a disease-associated mutation site in the three-dimensional structure of the cardiac muscle ryanodine receptor
    Zheng Liu
    Wadsworth Center, New York State Department of Health, Albany, 12201, USA
    J Biol Chem 280:37941-7. 2005
    The cardiac muscle ryanodine receptor (RyR2) functions as a calcium release channel in the heart. Up to 40 mutations in RyR2 have been linked to genetic forms of sudden cardiac death...
  19. pmc Ser-2030, but not Ser-2808, is the major phosphorylation site in cardiac ryanodine receptors responding to protein kinase A activation upon beta-adrenergic stimulation in normal and failing hearts
    Bailong Xiao
    Cardiovascular Research Group, Department of Physiology and Biophysics, University of Calgary, Calgary, Alberta, Canada
    Biochem J 396:7-16. 2006
    We have recently shown that RyR2 (cardiac ryanodine receptor) is phosphorylated by PKA (protein kinase A/cAMP-dependent protein kinase) at two major sites, Ser-2030 and Ser-2808...
  20. pmc Mice with the R176Q cardiac ryanodine receptor mutation exhibit catecholamine-induced ventricular tachycardia and cardiomyopathy
    Prince J Kannankeril
    Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN 37232, USA
    Proc Natl Acad Sci U S A 103:12179-84. 2006
    Mutations in the cardiac ryanodine receptor 2 (RyR2) have been associated with catecholaminergic polymorphic ventricular tachycardia and a form of arrhythmogenic right ventricular dysplasia...
  21. pmc Early cardiac hypertrophy in mice with impaired calmodulin regulation of cardiac muscle Ca release channel
    Naohiro Yamaguchi
    Department of Biochemistry and Biophysics, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA
    J Clin Invest 117:1344-53. 2007
    ..have shown that calmodulin (CaM) inhibits the activity of cardiac muscle cell Ca(2+) release channel ryanodine receptor 2 (RyR2)...
  22. pmc Arrhythmogenic mechanisms in a mouse model of catecholaminergic polymorphic ventricular tachycardia
    Marina Cerrone
    Department of Pharmacology, SUNY Upstate Medical University, Syracuse, NY 13210, USA
    Circ Res 101:1039-48. 2007
    ..Catecholaminergic polymorphic VT is caused by enhanced Ca2+ release through defective ryanodine receptor (RyR2) channels...
  23. pmc Physiological consequences of the P2328S mutation in the ryanodine receptor (RyR2) gene in genetically modified murine hearts
    C A Goddard
    Physiological Laboratory, University of Cambridge, Cambridge, UK
    Acta Physiol (Oxf) 194:123-40. 2008
    To explore the physiological consequences of the ryanodine receptor (RyR2)-P2328S mutation associated with catecholaminergic polymorphic ventricular tachycardia (CPVT).
  24. pmc Leaky Ca2+ release channel/ryanodine receptor 2 causes seizures and sudden cardiac death in mice
    Stephan E Lehnart
    Department of Physiology and Cellular Biophysics, Clyde and Helen Wu Center for Molecular Cardiology, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA
    J Clin Invest 118:2230-45. 2008
    The Ca2+ release channel ryanodine receptor 2 (RyR2) is required for excitation-contraction coupling in the heart and is also present in the brain...
  25. pmc Roles of IP3R and RyR Ca2+ channels in endoplasmic reticulum stress and beta-cell death
    Dan S Luciani
    Department of Cellular and Physiological Sciences, University of British Columbia, Vancouver, British Comlumbia, Canada
    Diabetes 58:422-32. 2009
    ..Here, we examined the effects of stimulating or inhibiting the ER-resident inositol trisphosphate receptors (IP(3)Rs) and the ryanodine receptors (RyRs) on the induction of beta-cell ER stress and apoptosis...
  26. pmc Increased Ca2+ sensitivity of the ryanodine receptor mutant RyR2R4496C underlies catecholaminergic polymorphic ventricular tachycardia
    Maria Fernandez-Velasco
    Institut National de la Sante et de la Recherche Medicale, U637, Universite de Montpellier, Montpellier, France
    Circ Res 104:201-9, 12p following 209. 2009
    Cardiac ryanodine receptor (RyR2) mutations are associated with autosomal dominant catecholaminergic polymorphic ventricular tachycardia, suggesting that alterations in Ca(2+) handling underlie this disease...
  27. pmc Calmodulin kinase II-mediated sarcoplasmic reticulum Ca2+ leak promotes atrial fibrillation in mice
    Mihail G Chelu
    Department of Molecular Physiology and Biophysics, Baylor College of Medicine, Houston, Texas, USA
    J Clin Invest 119:1940-51. 2009
    ..Here, we have shown that mice with a genetic gain-of-function defect in Ryr2 (which we termed Ryr2R176Q/+ mice) did not exhibit spontaneous AF but that rapid atrial pacing unmasked an ..
  28. pmc Catecholaminergic polymorphic ventricular tachycardia is caused by mutation-linked defective conformational regulation of the ryanodine receptor
    Hitoshi Uchinoumi
    Department of Medicine and Clinical Science, Division of Cardiology, Yamaguchi University Graduate School of Medicine, 1 1 1 Minamikogushi, Ube, Yamaguchi, 755 8505, Japan
    Circ Res 106:1413-24. 2010
    ..tachycardia (CPVT) is caused by a single point mutation in a well-defined region of the cardiac type 2 ryanodine receptor (RyR)2...
  29. pmc Ryanodine receptor phosphorylation by calcium/calmodulin-dependent protein kinase II promotes life-threatening ventricular arrhythmias in mice with heart failure
    Ralph J van Oort
    Department of Molecular Physiology and Biophysics, Baylor College of Medicine, One Baylor Plaza, BCM335, Houston, TX 77030, USA
    Circulation 122:2669-79. 2010
    ..Although abnormal Ca(2+) release from the sarcoplasmic reticulum through ryanodine receptors (RyR2) has been linked to arrhythmogenesis, the molecular mechanisms triggering release of arrhythmogenic Ca(2+) remain ..
  30. doi The deletion of exon 3 in the cardiac ryanodine receptor is rescued by β strand switching
    Paolo A Lobo
    Department of Biochemistry and Molecular Biology, University of British Columbia, 2350 Health Sciences Mall, room 2 320, Vancouver, BC V6T1Z3, Canada
    Structure 19:790-8. 2011
    Mutations in the cardiac Ryanodine Receptor (RYR2) are linked to triggered arrhythmias. Removal of exon 3 results in a severe form of catecholaminergic polymorphic ventricular tachycardia (CPVT)...
  31. pmc Carvedilol and its new analogs suppress arrhythmogenic store overload-induced Ca2+ release
    Qiang Zhou
    Libin Cardiovascular Institute of Alberta, Department of Physiology and Pharmacology, University of Calgary, Calgary, Alberta, Canada
    Nat Med 17:1003-9. 2011
    ..tested that effectively suppresses SOICR by directly reducing the open duration of the cardiac ryanodine receptor (RyR2)...
  32. pmc Inhibition of CaMKII phosphorylation of RyR2 prevents induction of atrial fibrillation in FKBP12.6 knockout mice
    Na Li
    Department of Molecular Physiology and Biophysics, Baylor College of Medicine, Houston, TX 77030, USA
    Circ Res 110:465-70. 2012
    ..Whereas increased Ca(2+)/calmodulin-dependent protein kinase II (CaMKII) activity has been proposed to contribute to SR leak and AF induction, downstream targets of CaMKII remain controversial...
  33. pmc Abnormal termination of Ca2+ release is a common defect of RyR2 mutations associated with cardiomyopathies
    Yijun Tang
    Department of Physiology and Pharmacology, The Libin Cardiovascular Institute of Alberta, University of Calgary, Calgary, AB, Canada
    Circ Res 110:968-77. 2012
    Naturally occurring mutations in the cardiac ryanodine receptor (RyR2) have been associated with both cardiac arrhythmias and cardiomyopathies...
  34. pmc Role of RyR2 phosphorylation at S2814 during heart failure progression
    Jonathan L Respress
    Department of Molecular Physiology and Biophysics, Baylor College of Medicine, Houston, TX 77030, USA
    Circ Res 110:1474-83. 2012
    ..However, the importance of CaMKII phosphorylation of ryanodine receptors (RyR2) in HF development and associated diastolic sarcoplasmic reticulum Ca(2+) leak is unclear.
  35. pmc Calcium leak through ryanodine receptors leads to atrial fibrillation in 3 mouse models of catecholaminergic polymorphic ventricular tachycardia
    Jian Shan
    Clyde and Helen Wu Center for Molecular Cardiology, Department of Physiology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA
    Circ Res 111:708-17. 2012
    ..The ryanodine receptor (RyR2) is the major calcium (Ca2+) release channel on the sarcoplasmic reticulum (SR) required for excitation-contraction ..
  36. pmc Cardiac ryanodine receptors control heart rate and rhythmicity in adult mice
    Michael J Bround
    Cardiovascular Research Group, Life Sciences Institute, University of British Columbia, 5358 Life Sciences Building, 2350 Health Sciences Mall, Vancouver, BC, Canada V6T 1Z3
    Cardiovasc Res 96:372-80. 2012
    ..While it is widely accepted that the type 2 ryanodine receptor (Ryr2) is the major Ca(2+) release channel in excitation-contraction coupling, the role of these ..
  37. pmc Role of leaky neuronal ryanodine receptors in stress-induced cognitive dysfunction
    Xiaoping Liu
    Department of Physiology and Cellular Biophysics, Clyde and Helen Wu Center for Molecular Cardiology, Columbia University College of Physicians and Surgeons, New York, NY 10032, USA
    Cell 150:1055-67. 2012
    The type 2 ryanodine receptor/calcium release channel (RyR2), required for excitation-contraction coupling in the heart, is abundant in the brain...
  38. pmc Heterogeneity of ryanodine receptor dysfunction in a mouse model of catecholaminergic polymorphic ventricular tachycardia
    Randall Loaiza
    Center for Arrhythmia Research, Cardiovascular Division, Department of Internal Medicine, University of Michigan, Ann Arbor, MI 48109, USA
    Circ Res 112:298-308. 2013
    Most cardiac ryanodine receptor (RyR2) mutations associated with catecholaminergic polymorphic ventricular tachycardia (CPVT) are postulated to cause a distinctive form of Ca(2+) release dysfunction...
  39. pmc Cardiomyocyte ATP production, metabolic flexibility, and survival require calcium flux through cardiac ryanodine receptors in vivo
    Michael J Bround
    Cardiovascular Research Group, Life Sciences Institute, University of British Columbia, Vancouver V6T 1Z3, Canada
    J Biol Chem 288:18975-86. 2013
    ..We report that controlled in vivo depletion of cardiac RYR2, using a conditional gene knock-out strategy (cRyr2KO mice), is sufficient to reduce mitochondrial Ca(2+) and ..
  40. pmc Phospholamban knockout breaks arrhythmogenic Ca²⁺ waves and suppresses catecholaminergic polymorphic ventricular tachycardia in mice
    Yunlong Bai
    Department of Physiology and Pharmacology, Libin Cardiovascular Institute of Alberta, University of Calgary, Calgary, Alberta, Canada
    Circ Res 113:517-26. 2013
    ..An important question is whether these seemingly paradoxical effects of PLN-KO exacerbate or protect against Ca²⁺-triggered arrhythmias...
  41. pmc Ca(2+)-induced Ca2+ release in myocytes from dyspedic mice lacking the type-1 ryanodine receptor
    H Takeshima
    Department of Neurochemistry, Tokyo Institute of Psychiatry, Japan
    EMBO J 14:2999-3006. 1995
    ..Although RyR-3 does not mediate excitation-contraction coupling of the skeletal muscle type, we propose that RyR-3 may induce intracellular Ca2+ release in response to a Ca2+ rise with a high threshold...
  42. ncbi Molecular identification of the ryanodine receptor pore-forming segment
    M Zhao
    Cardiovascular Research Group, Department of Physiology, University of Calgary, Calgary, Alberta T2N 4N1, Canada
    J Biol Chem 274:25971-4. 1999
    ..These data suggest that this conserved region constitutes an essential part of the ryanodine binding site and the channel conduction pathway of the ryanodine receptor...
  43. ncbi Enhanced store overload-induced Ca2+ release and channel sensitivity to luminal Ca2+ activation are common defects of RyR2 mutations linked to ventricular tachycardia and sudden death
    Dawei Jiang
    Department of Physiology and Biophysics, University of Calgary, Alberta, Canada
    Circ Res 97:1173-81. 2005
    Ventricular tachycardia (VT) is the leading cause of sudden death, and the cardiac ryanodine receptor (RyR2) is emerging as an important focus in its pathogenesis...
  44. ncbi Arrhythmogenesis in catecholaminergic polymorphic ventricular tachycardia: insights from a RyR2 R4496C knock-in mouse model
    Nian Liu
    Molecular Cardiology, IRCCS Fondazione Salvatore Maugeri, Pavia, Italy
    Circ Res 99:292-8. 2006
    ..disease characterized by life threatening arrhythmias and mutations in the gene encoding the ryanodine receptor (RyR2)...
  45. doi Glucocorticoid receptor is required for foetal heart maturation
    Eva A Rog-Zielinska
    Centre for Cardiovascular Science, University of Edinburgh, Queen s Medical Research Institute, Edinburgh EH16 4TJ, UK
    Hum Mol Genet 22:3269-82. 2013
    ....
  46. pmc Cardiac calcium signalling pathologies associated with defective calmodulin regulation of type 2 ryanodine receptor
    Juan José Arnáiz-Cot
    M Morad Cardiac Signaling Center, 173 Ashley Ave, Bioengineering Building, Room 306, Charleston, SC 29403, USA
    J Physiol 591:4287-99. 2013
    Cardiac ryanodine receptor (RyR2) is a homotetramer of 560 kDa polypeptides regulated by calmodulin (CaM), which decreases its open probability at diastolic and systolic Ca(2+) concentrations...
  47. pmc The ryanodine receptor store-sensing gate controls Ca2+ waves and Ca2+-triggered arrhythmias
    Wenqian Chen
    The Libin Cardiovascular Institute of Alberta, Department of Physiology and Pharmacology, University of Calgary, Calgary, Alberta, Canada
    Nat Med 20:184-92. 2014
    ..point mutation, E4872A, in the helix bundle crossing region (the proposed gate) of the cardiac ryanodine receptor (RyR2) completely abolishes luminal, but not cytosolic, Ca(2+) activation of RyR2...
  48. pmc Generation and characterization of a mouse model harboring the exon-3 deletion in the cardiac ryanodine receptor
    Yingjie Liu
    Libin Cardiovascular Institute of Alberta, Departments of Physiology and Pharmacology, and Biochemistry and Molecular Biology, University of Calgary, Calgary, Alberta, Canada
    PLoS ONE 9:e95615. 2014
    A large genomic deletion in human cardiac ryanodine receptor (RYR2) gene has been detected in a number of unrelated families with various clinical phenotypes, including catecholaminergic polymorphic ventricular tachycardia (CPVT)...
  49. doi The arrhythmogenic human HRC point mutation S96A leads to spontaneous Ca(2+) release due to an impaired ability to buffer store Ca(2+)
    Joe Z Zhang
    Department of Physiology and HeartOtago, Otago School of Medical Sciences, University of Otago, Dunedin 9054, New Zealand
    J Mol Cell Cardiol 74:22-31. 2014
    ..release events may be linked to store overload induced Ca(2+) release (SOICR) via the cardiac ryanodine receptor (RyR2)...
  50. ncbi Increase of type 2 ryanodine receptors in mouse nucleus accumbens in the development and expression of morphine-induced place preference
    Kazuhiro Kurokawa
    Department of Pharmacology, Kawasaki Medical School, Japan
    J Pharmacol Sci 126:285-90. 2014
    ..The expression of morphine-induced CPP was abolished by dantrolene administration before the post-conditioning test. These findings suggest that RyRs-2 in the NAcc participate in the development and expression of morphine-induced CPP. ..
  51. doi Palmitoyl-carnitine increases RyR2 oxidation and sarcoplasmic reticulum Ca2+ leak in cardiomyocytes: Role of adenine nucleotide translocase
    J Roussel
    Inserm U1046, UMR CNRS 9214, Universite de Montpellier, Montpellier, France
    Biochim Biophys Acta 1852:749-58. 2015
    ..In addition, PC increased type 2 ryanodine receptor (RyR2) oxidation, S-nitrosylation and dissociation of FKBP12...
  52. pmc Mitochondrial calcium overload is a key determinant in heart failure
    Gaetano Santulli
    Department of Physiology and Cellular Biophysics, College of Physicians and Surgeons, Columbia University Medical Center, New York, NY 10032 Helen and Clyde Wu Center for Molecular Cardiology, College of Physicians and Surgeons, Columbia University Medical Center, New York, NY 10032
    Proc Natl Acad Sci U S A 112:11389-94. 2015
    ..Using murine models harboring RyR2 mutations that either cause or inhibit SR Ca2+ leak, we found that leaky RyR2 channels result in mitochondrial Ca2+ ..
  53. pmc Ablation of HRC alleviates cardiac arrhythmia and improves abnormal Ca handling in CASQ2 knockout mice prone to CPVT
    Bin Liu
    Department of Physiology and Cell Biology, College of Medicine, and Davis Heart and Lung Research Institute, The Ohio State University, 473 W 12th Avenue, Columbus, OH 43210, USA
    Cardiovasc Res 108:299-311. 2015
    ..We hypothesized that given the apparent overlap of functions of CASQ2 and HRC, simultaneous ablation of both would deteriorate the cardiac phenotype compared with the single knockouts...
  54. pmc Calstabin 2: An important regulator for learning and memory in mice
    Qi Yuan
    National Laboratory of Biomacromolecules, Institute of Biophysics, Chinese Academy of Sciences, Beijing 100101, China
    Sci Rep 6:21087. 2016
    Calstabin2, also named FK506 binding protein 12.6 (FKBP12.6), is a subunit of ryanodine receptor subtype 2 (RyR2) macromolecular complex, which is an intracellular calcium channel and abundant in the brain...
  55. pmc Extensive Ca2+ leak through K4750Q cardiac ryanodine receptors caused by cytosolic and luminal Ca2+ hypersensitivity
    Akira Uehara
    Department of Physiology, Fukuoka University School of Medicine, Fukuoka 814 0180, Japan
    J Gen Physiol . 2017
    Various ryanodine receptor 2 (RyR2) point mutations cause catecholamine-induced polymorphic ventricular tachycardia (CPVT), a life-threatening arrhythmia evoked by diastolic intracellular Ca2+ release dysfunction...
  56. pmc Ablation of triadin causes loss of cardiac Ca2+ release units, impaired excitation-contraction coupling, and cardiac arrhythmias
    Nagesh Chopra
    Division of Clinical Pharmacology, Department of Medicine, Vanderbilt University, Nashville, TN 37232, USA
    Proc Natl Acad Sci U S A 106:7636-41. 2009
    ..2) triggers Ca(2+) release from juxtaposed Ca(2+) release channels (RyR2) located in junctional sarcoplasmic reticulum (jSR)...
  57. pmc Mutation-linked defective interdomain interactions within ryanodine receptor cause aberrant Ca²⁺release leading to catecholaminergic polymorphic ventricular tachycardia
    Takeshi Suetomi
    Department of Medicine and Clinical Science, Division of Cardiology, Yamaguchi University Graduate School of Medicine, Yamaguchi, 755 8505, Japan
    Circulation 124:682-94. 2011
    ..ventricular tachycardia is induced by single amino acid mutations within the cardiac ryanodine receptor (RyR2) remains elusive...
  58. pmc In situ confocal imaging in intact heart reveals stress-induced Ca(2+) release variability in a murine catecholaminergic polymorphic ventricular tachycardia model of type 2 ryanodine receptor(R4496C+/-) mutation
    Biyi Chen
    Division of Cardiovascular Medicine, Department of Internal Medicine, University of Iowa Carver College of Medicine, Iowa City, IA 52242, USA
    Circ Arrhythm Electrophysiol 5:841-9. 2012
    Catecholaminergic polymorphic ventricular tachycardia is directly linked to mutations in proteins (eg, type 2 ryanodine receptor [RyR2](R4496C)) responsible for intracellular Ca(2+) homeostasis in the heart...
  59. pmc Decreased RyR2 refractoriness determines myocardial synchronization of aberrant Ca2+ release in a genetic model of arrhythmia
    Lucia Brunello
    D Davis Heart and Lung Research Institute, College of Medicine, The Ohio State University, Columbus, OH 43210, USA
    Proc Natl Acad Sci U S A 110:10312-7. 2013
    ..Our study reveals how aberrant DCR events can become synchronized in the intact myocardium, leading to triggered activity and the resultant DCs in the settings of a cardiac rhythm disorder...
  60. pmc The organization of proteins in the human red blood cell membrane. A review
    T L Steck
    J Cell Biol 62:1-19. 1974
  61. ncbi Regulation of mouse egg activation: presence of ryanodine receptors and effects of microinjected ryanodine and cyclic ADP ribose on uninseminated and inseminated eggs
    T Ayabe
    Department of Obstetrics and Gynecology, University of Pennsylvania, Philadelphia 19104, USA
    Development 121:2233-44. 1995
    ..Results of these experiments suggest that although ryanodine receptors are present and functional, release of Ca2+ from this store is not essential for sperm-induced egg activation...
  62. ncbi Tissue-specific alternative splicing of mouse brain type ryanodine receptor/calcium release channel mRNA
    R Miyatake
    Department of Neuropsychiatry, Kagawa Medical University, Kita gun, Japan
    FEBS Lett 395:123-6. 1996
    ..However, interestingly, the splicing type (RyR3-I) mRNA was detected only in the cerebrum. These findings suggest that the splicing variants RyR3-I and RyR3-II may generate functional differences of RyR3 in a tissue-specific manner...
  63. ncbi The physical and genetic map surrounding the Lyst gene on mouse chromosome 13
    D J Misumi
    Millennium Pharmaceuticals, Inc, Cambridge, Massachusetts 02139, USA
    Genomics 40:147-50. 1997
    ..These data are consistent with LYST being the gene for the human Chediak-Higashi Syndrome and strengthen the synteny relationship between MMU13 and human 1q43...
  64. ncbi Expression of the ryanodine receptor type 3 calcium release channel during development and differentiation of mammalian skeletal muscle cells
    P Tarroni
    Dipartimento di Ricerca Biologica e Tecnologica DIBIT, San Raffaele Scientific Institute, Via Olgettina 58, 20132 Milan, Italy
    J Biol Chem 272:19808-13. 1997
    ..These findings indicate that RyR3 is preferentially expressed in differentiated skeletal muscle cells. In addition, during skeletal muscle development, its expression is regulated differently from that of RyR1...
  65. ncbi Intracellular calcium release channel expression during embryogenesis
    N Rosemblit
    Molecular Cardiology Program, Divisions of Circulatory Physiology and Cardiology, Department of Medicine, New York, New York, 10032, USA
    Dev Biol 206:163-77. 1999
    ....
  66. ncbi Genetic and physical delineation of the region overlapping the progressive motor neuropathy (pmn) locus on mouse chromosome 13
    N Martin
    Unite de Genetique des Mammiferes, Institut Pasteur, 25 rue du Docteur Roux, F 75724 Paris Cedex 15, France
    Genomics 75:9-16. 2001
    ....
  67. ncbi Developmental expression of the calcium release channels during early neurogenesis of the mouse cerebral cortex
    A V Faure
    Laboratoire Canaux Ioniques et Signalisation, INSERM E 9931, Departement de Biologie Moleculaire et Structurale, 17 rue des Martyrs, F 38054 Grenoble, France
    Eur J Neurosci 14:1613-22. 2001
    ..Altogether, they provide a molecular basis for analyzing the developmental modulation of calcium signals useful for neurogenesis progression...
  68. ncbi Transgenic overexpression of the Ca2+-binding protein S100A1 in the heart leads to increased in vivo myocardial contractile performance
    Patrick Most
    Medizinische Universitätsklinik und Poliklinik III, Universität zu Heidelberg, 69115 Heidelberg
    J Biol Chem 278:33809-17. 2003
    ..Because S100A1 protein expression is down-regulated in heart failure, increasing S100A1 expression in the heart may represent a novel means to augment contractility...
  69. pmc Dilated cardiomyopathy caused by tissue-specific ablation of SC35 in the heart
    Jian Hua Ding
    Department of Cellular and Molecular Medicine, University of California at San Diego, La Jolla, CA 92093 0651, USA
    EMBO J 23:885-96. 2004
    ..allowed the identification of downregulated genes by microarray, one of which was the cardiac-specific ryanodine receptor 2. We showed that downregulation of this critical Ca2+ release channel preceded disease symptoms and that the ..
  70. ncbi Decreased expression of ryanodine receptors alters calcium-induced calcium release mechanism in mdx duodenal myocytes
    Jean Luc Morel
    Laboratoire de Signalisation et Interactions Cellulaires, CNRS UMR 5017, Universite de Bordeaux II, 146 rue Leo Saignat, 33076 Bordeaux Cedex, France
    J Biol Chem 279:21287-93. 2004
    ..depolarization-induced calcium responses were dose-dependently and specifically inhibited with the anti-type 2 ryanodine receptor antibody...
  71. pmc RYR2 proteins contribute to the formation of Ca(2+) sparks in smooth muscle
    Guangju Ji
    Department of Biomedical Sciences, Cornell University, Ithaca, NY 14853 6401, USA
    J Gen Physiol 123:377-86. 2004
    ..FKBP12.6 proteins interact with RYR2 Ca(2+) release channels and the absence of these proteins predictably alters the amplitude and kinetics of RYR2 ..
  72. ncbi Ryanodine receptors are expressed and functionally active in mouse spermatogenic cells and their inhibition interferes with spermatogonial differentiation
    Pieranna Chiarella
    Department of Histology and Medical Embryology and Centro di Eccellenza Biologia e Medicina Molecolare, University of Rome La Sapienza, Via A Scarpa 14, 00161 Roma, Italy
    J Cell Sci 117:4127-34. 2004
    ..RyR1 was expressed in spermatogonia, pachytene spermatocytes and round spermatids whereas RyR2 was found only in 5- to 10-day-old testis but not in germ cells...
  73. ncbi Complementary DNA cloning, genomic characterization and expression analysis of a mammalian gene encoding histidine-rich calcium binding protein
    Sunghee Hong
    Department of Life Science, Gwangju Institute of Science and Technology, Gwangju 500 712, Republic of Korea
    Biochim Biophys Acta 1727:188-96. 2005
    ..Collectively, our study provides comprehensive information about the structure and expression of the mammalian HRC gene, together with the comparative expression data of the related SR genes...
  74. ncbi Crucial role of the sarcoplasmic reticulum in the developmental regulation of Ca2+ transients and contraction in cardiomyocytes derived from embryonic stem cells
    Ji Dong Fu
    Laboratory of Molecular Cardiology of Institute of Health Sciences, Shanghai Institutes for Biological Sciences SIBS, Chinese Academy of Sciences CAS and Shanghai Jiao Tong University School of Medicine, Shanghai, China
    FASEB J 20:181-3. 2006
    ..excitation-contraction coupling is critically regulated by sarcoplasmic reticulum (SR) Ca2+ release via type 2 ryanodine receptor (RyR2), but generally, it is believed that SR-function is rudimentary in the fetal heart and in ..
  75. pmc Analysis of calstabin2 (FKBP12.6)-ryanodine receptor interactions: rescue of heart failure by calstabin2 in mice
    Fannie Huang
    Clyde and Helen Wu Center for Molecular Cardiology, Department of Physiology and Cellular Biophysics, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA
    Proc Natl Acad Sci U S A 103:3456-61. 2006
    ..b>RyR2, the predominant isoform in cardiac myocytes, comprises a macromolecular complex that includes calstabin2 (FKBP12...
  76. pmc Sox17 is essential for the specification of cardiac mesoderm in embryonic stem cells
    Yu Liu
    Center for Cardiovascular Development, Baylor College of Medicine, Houston, TX 77030, USA
    Proc Natl Acad Sci U S A 104:3859-64. 2007
    ..Sox17 short-hairpin RNA blocks cardiac myogenesis non-cell autonomously and impairs the induction of Hex, a homeodomain transcription factor that is known to be required for the production of endoderm-derived heart-inducing factors...
  77. pmc Ryanodine receptor type 2 deficiency changes excitation-contraction coupling and membrane potential in urinary bladder smooth muscle
    Shingo Hotta
    Department of Molecular and Cellular Pharmacology, Graduate School of Pharmaceutical Science, Nagoya City University, 3 1 Tanabedori, Mizuhoku, Nagoya 467 8603, Japan
    J Physiol 582:489-506. 2007
    The possibility that the ryanodine receptor type 2 (RyR2) can function as the major Ca(2+)-induced Ca(2+) release (CICR) channel in excitation-contraction (E-C) coupling was examined in smooth muscle cells (SMCs) isolated from urinary ..
  78. pmc Regulation of ryanodine receptor-dependent calcium signaling by polycystin-2
    Georgia I Anyatonwu
    Department of Pharmacology, Yale University School of Medicine, New Haven, CT 06520 8066, USA
    Proc Natl Acad Sci U S A 104:6454-9. 2007
    ..Here, we show that PC2 coimmunoprecipitates with the cardiac ryanodine receptor (RyR2) from mouse heart...
  79. ncbi ADP-ribosyl cyclase and ryanodine receptor activity contribute to basal renal vasomotor tone and agonist-induced renal vasoconstriction in vivo
    Tiffany L Thai
    Department of Cell and Molecular Physiology, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA
    Am J Physiol Renal Physiol 293:F1107-14. 2007
    ....
  80. ncbi Interaction of calcium/calmodulin-dependent protein kinase IIdeltaC with sorcin indirectly modulates ryanodine receptor function in cardiac myocytes
    Diana F Anthony
    Division of Physiology and Pharmacology, Strathclyde Institute of Pharmacy and Biomedical Sciences, University of Strathclyde, Glasgow G4 ONR, UK
    J Mol Cell Cardiol 43:492-503. 2007
    ..Since both proteins are capable of directly modulating RyR(2) activity, this interaction may serve as an additional or alternative indirect route by which both proteins can regulate RyR(2) opening status in cardiac myocytes...
  81. ncbi Intact beta-adrenergic response and unmodified progression toward heart failure in mice with genetic ablation of a major protein kinase A phosphorylation site in the cardiac ryanodine receptor
    Nancy A Benkusky
    Department of Physiology, University of Wisconsin, Madison, USA
    Circ Res 101:819-29. 2007
    ..persistent activation of the sympathetic system during chronic stress leads to PKA "hyperphosphorylation" of RyR2-S2808, which increases Ca2+ release by augmenting the sensitivity of the RyR2 channel to diastolic Ca2+...
  82. pmc Removal of FKBP12.6 does not alter the conductance and activation of the cardiac ryanodine receptor or the susceptibility to stress-induced ventricular arrhythmias
    Jianmin Xiao
    Libin Cardiovascular Institute of Alberta, Department of Physiology and Biophysics, University of Calgary, Calgary, AB, T2N 4N1, Canada
    J Biol Chem 282:34828-38. 2007
    The 12.6-kDa FK506-binding protein (FKBP12.6) is considered to be a key regulator of the cardiac ryanodine receptor (RyR2), but its precise role in RyR2 function is complex and controversial...
  83. doi A glutathione deficit alters dopamine modulation of L-type calcium channels via D2 and ryanodine receptors in neurons
    Pascal Steullet
    Center for Psychiatric Neurosciences, Centre Hospitalier Universitaire Vaudois and University of Lausanne, Prilly Lausanne, Switzerland
    Free Radic Biol Med 44:1042-54. 2008
    ..This represents a mechanism by which dopaminergic systems could be dysfunctional under conditions of impaired glutathione synthesis as in schizophrenia...
  84. pmc ARVC-related mutations in divergent region 3 alter functional properties of the cardiac ryanodine receptor
    Andrea Koop
    Institut fur Physiologische Chemie, Abteilung Biochemie Supramolekularer Systeme, Ruhr Universitat Bochum, 44780 Bochum, Germany
    Biophys J 94:4668-77. 2008
    Two single-nucleotide polymorphisms in the type 2 ryanodine receptor (RyR2) leading to the nonsynonymous amino acid replacements G1885E and G1886S are associated with arrhythmogenic right ventricular cardiomyopathy in patients who are ..
  85. pmc Adrenergic regulation of cardiac contractility does not involve phosphorylation of the cardiac ryanodine receptor at serine 2808
    Scott M MacDonnell
    Department of Physiology, Cardiovascular Research Center, Temple University School of Medicine, Philadelphia, PA, USA
    Circ Res 102:e65-72. 2008
    ..These results show that protein kinase A phosphorylation of ryanodine receptor Ser2808 does not have a major role in sympathetic nervous system regulation of normal cardiac function...
  86. doi Type 1 and type 3 ryanodine receptors are selectively involved in muscarinic antinociception in mice: an antisense study
    N Galeotti
    Department of Preclinical and Clinical Pharmacology, Viale G Pieraccini 6, I 50139 Florence, Italy
    Neuroscience 153:814-22. 2008
    ..A selective involvement of RyR1 and RyR3 in supraspinal muscarinic analgesia was demonstrated whereas RyR2 appears not to play an essential role in acute thermal and inflammatory pain.
  87. doi A gene-specific cerebral types 1, 2, and 3 RyR protein knockdown induces an antidepressant-like effect in mice
    Nicoletta Galeotti
    Department of Preclinical and Clinical Pharmacology, Viale G Pieraccini, Florence, Italy
    J Neurochem 106:2385-94. 2008
    ..These findings identify cerebral RyRs as critical targets underlying depressive states and should facilitate the comprehension of the pathophysiology of mood disorders and help developing of new therapeutical strategies...
  88. doi Hypoxia differently modulates gene expression of inositol 1,4,5-trisphosphate receptors in mouse kidney and HEK 293 cell line
    Dana Jurkovicova
    Institute of Molecular Physiology and Genetics, Centre of Excellence for Cardiovascular Research SAS, Bratislava, Slovakia
    Ann N Y Acad Sci 1148:421-7. 2008
    ..mRNA for inositol 1,4,5-trisphosphate receptors (IP3R) type 1 (IP3R1) and ryanodine receptors (RyR) type 2 (RyR2), in culture of HEK 293 cells the gene expression of all IP3Rs decreased without affecting viability of the cells...
  89. pmc Membrane depolarization causes a direct activation of G protein-coupled receptors leading to local Ca2+ release in smooth muscle
    Qing Hua Liu
    Center for Cardiovascular Sciences, Albany Medical College, Albany, NY 12208, USA
    Proc Natl Acad Sci U S A 106:11418-23. 2009
    ..IP(3)), inducing initial Ca(2+) release through IP(3) receptors and then further Ca(2+) release via RyR2 due to a local Ca(2+)-induced Ca(2+) release process...
  90. pmc Dissociation of FKBP12.6 from ryanodine receptor type 2 is regulated by cyclic ADP-ribose but not beta-adrenergic stimulation in mouse cardiomyocytes
    Xu Zhang
    National Laboratory of Biomacromolecules, Institute of Biophysics, Chinese Academy of Sciences, 15 Datun Road, Chaoyang District, Beijing 100101, China
    Cardiovasc Res 84:253-62. 2009
    ..6 (FKBP12.6) regulatory proteins from ryanodine receptors subtype 2 (RYR2). We used FKBP12.6 null mice to test the extent to which the dissociation of FKBP12...
  91. pmc Intra-sarcoplasmic reticulum Ca2+ oscillations are driven by dynamic regulation of ryanodine receptor function by luminal Ca2+ in cardiomyocytes
    Sarah C W Stevens
    Department of Physiology and Cell Biology, Ohio State University, Columbus, OH 43214, USA
    J Physiol 587:4863-72. 2009
    During the cardiac cycle, the release of Ca(2+) from the sarcoplasmic reticulum (SR) through the ryanodine receptor (RyR2) channel complex is controlled by the levels of cytosolic and luminal Ca(2+) and alterations in these regulatory ..
  92. pmc Defective calmodulin binding to the cardiac ryanodine receptor plays a key role in CPVT-associated channel dysfunction
    Xiaojuan Xu
    Department of Medicine and Clinical Science, Division of Cardiology, Yamaguchi University Graduate School of Medicine, 1 1 1 Minamikogushi, Ube, Yamaguchi 755 8505, Japan
    Biochem Biophys Res Commun 394:660-6. 2010
    Calmodulin (CaM), one of the accessory proteins of the cardiac ryanodine receptor (RyR2), is known to play a significant role in the channel regulation of the RyR2...
  93. pmc Kinetics of FKBP12.6 binding to ryanodine receptors in permeabilized cardiac myocytes and effects on Ca sparks
    Tao Guo
    Department of Pharmacology, University of California, Davis, 451 Health Science Dr, Davis, CA 95616, USA
    Circ Res 106:1743-52. 2010
    FK506-binding proteins FKBP12.6 and FKBP12 are associated with cardiac ryanodine receptors (RyR2), and cAMP-dependent protein kinase A (PKA)-dependent phosphorylation of RyR2 was proposed to interrupt FKBP12...
  94. doi A calcium-induced calcium release mechanism supports luteinizing hormone-induced testosterone secretion in mouse Leydig cells
    Roberta Ribeiro Costa
    Department of Physiology, School of Medicine of Ribeirao Preto University of Sao Paulo, 14049 900 Ribeirao Preto, Sao Paulo, Brazil
    Am J Physiol Cell Physiol 299:C316-23. 2010
    ..These results not only broaden our understanding of the role played by calcium in Leydig cells but also show, for the first time, that RyRs have an important role in determining testosterone secretion by the testis...
  95. pmc Purkinje cells from RyR2 mutant mice are highly arrhythmogenic but responsive to targeted therapy
    Guoxin Kang
    The Leon H Charney Division of Cardiology, New York University School of Medicine, New York, NY 10016, USA
    Circ Res 107:512-9. 2010
    ..The Purkinje fiber network has been proposed as the source of arrhythmogenic Ca(2+) release events in catecholaminergic polymorphic ventricular tachycardia (CPVT), yet evidence supporting this mechanism at the cellular level is lacking...
  96. pmc Role of chronic ryanodine receptor phosphorylation in heart failure and β-adrenergic receptor blockade in mice
    Jian Shan
    Clyde and Helen Wu Center for Molecular Cardiology, Department of Physiology and Cellular Biophysics, Columbia University, New York, New York, USA
    J Clin Invest 120:4375-87. 2010
    Increased sarcoplasmic reticulum (SR) Ca2+ leak via the cardiac ryanodine receptor/calcium release channel (RyR2) is thought to play a role in heart failure (HF) progression. Inhibition of this leak is an emerging therapeutic strategy...
  97. pmc Inhibition of cardiac Ca2+ release channels (RyR2) determines efficacy of class I antiarrhythmic drugs in catecholaminergic polymorphic ventricular tachycardia
    Hyun Seok Hwang
    Oates Institute for Experimental Therapeutics, Vanderbilt University School of Medicine, Division of Clinical Pharmacology, Nashville, TN 37232 0575, USA
    Circ Arrhythm Electrophysiol 4:128-35. 2011
    ..polymorphic ventricular tachycardia (CPVT) is caused by mutations in the cardiac ryanodine receptor (RyR2) or calsequestrin (Casq2) and can be difficult to treat...
  98. pmc Dysfunctional ryanodine receptor and cardiac hypertrophy: role of signaling molecules
    Naohiro Yamaguchi
    Dept of Biochemistry and Biophysics, Univ of North Carolina, Chapel Hill, NC 27599 7260, USA
    Am J Physiol Heart Circ Physiol 300:H2187-95. 2011
    Mice with three amino acid mutations in the calmodulin binding domain of type-2 ryanodine receptor ion channel (Ryr2(ADA/ADA) mice) have impaired intracellular Ca(2+) handling and cardiac hypertrophy with death at an early age...
  99. ncbi Chromosomal localization of murine ryanodine receptor genes RYR1, RYR2, and RYR3 by in situ hybridization
    M G Mattei
    Unité INSERM 242, Hopital d Enfants de la Timone, Marseille, France
    Genomics 22:202-4. 1994
    ..the location on murine chromosomes of the three genes of the ryanodine receptor/Ca2+ channel family: RYR1, RYR2, and RYR3...
  100. ncbi Sequential docking, molecular differentiation, and positioning of T-Tubule/SR junctions in developing mouse skeletal muscle
    H Takekura
    Department of Physiological Sciences, National Institute of Fitness and Sports, Kanoya, Kagoshima, 891 2393, Japan
    Dev Biol 239:204-14. 2001
    ..These sequential stages suggests an order of inductive processes for the molecular differentiation and structural organization of the CRUs in skeletal muscle development...
  101. ncbi Enhanced basal activity of a cardiac Ca2+ release channel (ryanodine receptor) mutant associated with ventricular tachycardia and sudden death
    Dawei Jiang
    Cardiovascular Research Group, Department of Physiology and Biophysics, University of Calgary, Calgary, Alberta, Canada
    Circ Res 91:218-25. 2002
    Mutations in the human cardiac Ca2+ release channel (ryanodine receptor, RyR2) gene have recently been shown to cause effort-induced ventricular arrhythmias...

Research Grants6

  1. MOLECULAR CORRELATES OF HUMAN CA2+ CHANNEL REGULATION
    Martin Morad; Fiscal Year: 2003
    ....
  2. CARDIAC AND SKELETAL MUSCLE CALCIUM RELEASE CHANNELS
    Andrew Marks; Fiscal Year: 2009
    ..Our preliminary data suggest that the effect of PKA phosphorylation of RyR2 on FKBP12.6 binding is mediated by electrostatic repulsion due to negative charges on FKBP12...
  3. Regulation of cardiac ion channel function via allosteric modulators
    Andrew Marks; Fiscal Year: 2007
    ..6 (calstabin1/2). Four aims are proposed: Aim 1: Allosteric regulation of RyR2 by small molecules that enhance binding of calstabin2 to RyR2...
  4. Heterogeneity of hypoxic Ca2+ release in pulmonary and *
    Yong Xiao Wang; Fiscal Year: 2006
    ..Our preliminary studies, together with previous findings, suggest that specific RyR subtypes (RyR1, RyR2 and RyR3) are differentially expressed in the vasculature...
  5. Novel Signaling for Ca2+ and Release in Airway Myocytes
    Yong Xiao Wang; Fiscal Year: 2007
    ..6, and/or RyRs are ubiquitously expressed in a variety of cell types, the mechanistic findings from the proposed study will have general biological and pathological significance for various Ca2+-mediated cellular effects. ..
  6. Mechanisms for hypoxic Ca2+ release in pulmonary artery myocytes
    Yong Xiao Wang; Fiscal Year: 2010
    ..The findings from this proposal will enhance our understanding of cellular molecular mechanisms for hypoxic [Ca2+]i rise in PASMCs and associated HPV, and may lead to identify potential novel targets to treat pulmonary hypertension. ..