Ryr1

Summary

Gene Symbol: Ryr1
Description: ryanodine receptor 1
Alias: RYR-1, Ryr1l, ryanodine receptor 1, LOW QUALITY PROTEIN: ryanodine receptor 1, ryanodine receptor 1 (skeletal), ryanodine receptor 1, skeletal muscle, ryanodine receptor 1-like, skeletal muscle, skeletal muscle calcium release channel, skeletal muscle ryanodine receptor, skeletal muscle-type ryanodine receptor, type 1 ryanodine receptor
Species: rat
Products:     Ryr1

Top Publications

  1. Takeshima H, Iino M, Takekura H, Nishi M, Kuno J, Minowa O, et al. Excitation-contraction uncoupling and muscular degeneration in mice lacking functional skeletal muscle ryanodine-receptor gene. Nature. 1994;369:556-9 pubmed
    ..We report here mice with a targeted mutation in the skeletal muscle ryanodine receptor gene...
  2. Wang M, Hao L, Guo F, Zhong B, Zhong X, Yuan J, et al. Decreased intracellular [Ca2+ ] coincides with reduced expression of Dhpr?1s, RyR1, and diaphragmatic dysfunction in a rat model of sepsis. Muscle Nerve. 2017;56:1128-1136 pubmed publisher
    ..and co-localization of relevant calcium channels [dihydropyridine receptors, (DHPR?1s) and ryanodine receptors (RyR1)] remain unclear during sepsis...
  3. Kaya L, Meissner B, Riedl M, Muik M, Schwarzer C, Ferraguti F, et al. Direct association of the reticulon protein RTN1A with the ryanodine receptor 2 in neurons. Biochim Biophys Acta. 2013;1833:1421-33 pubmed publisher
    ..In a functional assay, RTN1A significantly reduced RyR2-mediated Ca(2+) oscillations. Thus, RTN1A and RyR2 might act as functional partners in the regulation of cytosolic Ca(2+) dynamics the in neurons. ..
  4. Lesiak A, Zhu M, Chen H, Appleyard S, Impey S, Lein P, et al. The environmental neurotoxicant PCB 95 promotes synaptogenesis via ryanodine receptor-dependent miR132 upregulation. J Neurosci. 2014;34:717-25 pubmed publisher
    ..In light of recent evidence implicating miR132 dysregulation in Rett syndrome and schizophrenia, these findings identify NDL PCBs as potential environmental risk factors for neurodevelopmental disorders. ..
  5. Kraner S, Wang Q, Novak K, Cheng D, Cool D, Peng J, et al. Upregulation of the CaV 1.1-ryanodine receptor complex in a rat model of critical illness myopathy. Am J Physiol Regul Integr Comp Physiol. 2011;300:R1384-91 pubmed publisher
    ..1 (DHPR/Ca(V) 1.1). Immunostaining revealed a subset of fibers with elevation of RYR1 and Ca(V) 1.1 that had severe atrophy and disorganization of sarcomeres...
  6. Samso M, Wagenknecht T. Apocalmodulin and Ca2+-calmodulin bind to neighboring locations on the ryanodine receptor. J Biol Chem. 2002;277:1349-53 pubmed
    Calmodulin (CaM) binds to the ryanodine receptor/calcium release channel of skeletal muscle (RyR1), both in the absence and presence of Ca(2+), and regulates the activity of the channel activity by activating and inhibiting it, ..
  7. Romero N, Monnier N, Viollet L, Cortey A, Chevallay M, Leroy J, et al. Dominant and recessive central core disease associated with RYR1 mutations and fetal akinesia. Brain. 2003;126:2341-9 pubmed
    ..Molecular genetic investigations led to the identification of mutations in the ryanodine receptor (RYR1) gene in three families, two with autosomal recessive (AR) and one with autosomal dominant (AD) inheritance...
  8. O Uchi J, Jhun B, Hurst S, Bisetto S, Gross P, Chen M, et al. Overexpression of ryanodine receptor type 1 enhances mitochondrial fragmentation and Ca2+-induced ATP production in cardiac H9c2 myoblasts. Am J Physiol Heart Circ Physiol. 2013;305:H1736-51 pubmed publisher
    ..We have previously reported that skeletal muscle-type ryanodine receptor (RyR) type 1 (RyR1), which expressed at the mitochondrial inner membrane, serves as an additional Ca(2+) uptake pathway in ..
  9. Duke A, Steele D. The presence of a functional t-tubule network increases the sensitivity of RyR1 to agonists in skinned rat skeletal muscle fibres. Cell Calcium. 2008;44:411-21 pubmed
    ..Longus (EDL) rat fibres were used as a model to study the influence of functional t-tubules on the properties of RyR1 in adult skeletal muscle. Fibres were superfused with solutions approximating to the intracellular milieu...

More Information

Publications51

  1. Rosker C, Meur G, Taylor E, Taylor C. Functional ryanodine receptors in the plasma membrane of RINm5F pancreatic beta-cells. J Biol Chem. 2009;284:5186-94 pubmed publisher
    ..Using RNAi selectively to reduce expression of RyR1, RyR2, or IP(3)R3, we showed that RyR2 mediates both the Ca(2+) entry and the plasma membrane currents evoked by ..
  2. Zvaritch E, Depreux F, Kraeva N, Loy R, Goonasekera S, Boncompagni S, et al. An Ryr1I4895T mutation abolishes Ca2+ release channel function and delays development in homozygous offspring of a mutant mouse line. Proc Natl Acad Sci U S A. 2007;104:18537-42 pubmed
    A heterozygous Ile4898 to Thr (I4898T) mutation in the human type 1 ryanodine receptor/Ca(2+) release channel (RyR1) leads to a severe form of central core disease...
  3. Rezgui S, Vassilopoulos S, Brocard J, Platel J, Bouron A, Arnoult C, et al. Triadin (Trisk 95) overexpression blocks excitation-contraction coupling in rat skeletal myotubes. J Biol Chem. 2005;280:39302-8 pubmed
    ..Thus, the level of Trisk 95 and not Trisk 51 is important on regulating the calcium release complex, and an excess of this protein can lead to an inhibition of the physiological function of the complex. ..
  4. Boschek C, Jones T, Smallwood H, Squier T, Bigelow D. Loss of the calmodulin-dependent inhibition of the RyR1 calcium release channel upon oxidation of methionines in calmodulin. Biochemistry. 2008;47:131-42 pubmed
    ..investigated the possible oxidation of CaM in skeletal muscle and its effect on the CaM-dependent regulation of the RyR1 calcium release channel...
  5. Jiang L, Wang Y, Zhang C, Sun Y. [Activity of adenosine triphosphatase and the expression of ryanodine receptor 1 mRNA in local tissue of pressure ulcer at early stage in gracilis of rats]. Zhonghua Shao Shang Za Zhi. 2011;27:178-204 pubmed
    To investigate changes in adenosine triphosphatase (ATPase) activity and expression of ryanodine receptor 1 (RyR1) mRNA in formation of pressure ulcer at early stage, and to analyze its mechanism...
  6. Monnier N, Kozak Ribbens G, Krivosic Horber R, Nivoche Y, Qi D, Kraev N, et al. Correlations between genotype and pharmacological, histological, functional, and clinical phenotypes in malignant hyperthermia susceptibility. Hum Mutat. 2005;26:413-25 pubmed
    ..Mutations associated with MHS have been reported in two genes: RYR1 and CACNA1S...
  7. Ryan T, Sharma P, Ignatchenko A, MacLennan D, Kislinger T, Gramolini A. Identification of novel ryanodine receptor 1 (RyR1) protein interaction with calcium homeostasis endoplasmic reticulum protein (CHERP). J Biol Chem. 2011;286:17060-8 pubmed publisher
    The ryanodine receptor type 1 (RyR1) is a homotetrameric Ca(2+) release channel located in the sarcoplasmic reticulum of skeletal muscle where it plays a role in the initiation of skeletal muscle contraction...
  8. Jona I, Szegedi C, Sarkozi S, Szentesi P, Csernoch L, Kovacs L. Altered inhibition of the rat skeletal ryanodine receptor/calcium release channel by magnesium in the presence of ATP. Pflugers Arch. 2001;441:729-38 pubmed
    ..At a low calcium concentration, the RyR had two ATP-binding sites with half-activatory concentrations of 19 and 350 microM, respectively. ..
  9. Takekura H, Flucher B, Franzini Armstrong C. Sequential docking, molecular differentiation, and positioning of T-Tubule/SR junctions in developing mouse skeletal muscle. Dev Biol. 2001;239:204-14 pubmed
    ..These sequential stages suggests an order of inductive processes for the molecular differentiation and structural organization of the CRUs in skeletal muscle development. ..
  10. Feng W, Tu J, Yang T, Vernon P, Allen P, Worley P, et al. Homer regulates gain of ryanodine receptor type 1 channel complex. J Biol Chem. 2002;277:44722-30 pubmed
    ..Here, we demonstrate that Homer proteins also physically associate with ryanodine receptors type 1 (RyR1) and regulate gating responses to Ca(2+), depolarization, and caffeine...
  11. Cheng L, Lu N, Zhang Y, Zhao Z. Ryanodine receptors contribute to the induction of nociceptive input-evoked long-term potentiation in the rat spinal cord slice. Mol Pain. 2010;6:1 pubmed publisher
    ..These data indicate that activation of presynaptic RyRs play a crucial role in the induction of LTP in the spinal pain pathway, probably through enhancement of transmitter release. ..
  12. Lanner J, Georgiou D, Joshi A, Hamilton S. Ryanodine receptors: structure, expression, molecular details, and function in calcium release. Cold Spring Harb Perspect Biol. 2010;2:a003996 pubmed publisher
    ..RyRs are the largest known ion channels (> 2MDa) and exist as three mammalian isoforms (RyR 1-3), all of which are homotetrameric proteins that interact with and are regulated by phosphorylation, redox ..
  13. Ryu S, Beutner G, Kinnally K, Dirksen R, Sheu S. Single channel characterization of the mitochondrial ryanodine receptor in heart mitoplasts. J Biol Chem. 2011;286:21324-9 pubmed publisher
  14. Al Qusairi L, Prokic I, Amoasii L, Kretz C, Messaddeq N, Mandel J, et al. Lack of myotubularin (MTM1) leads to muscle hypotrophy through unbalanced regulation of the autophagy and ubiquitin-proteasome pathways. FASEB J. 2013;27:3384-94 pubmed publisher
    ..These data support the hypothesis that the unbalanced regulation of the ubiquitin proteasome pathway and the autophagy machinery is a primary cause of the XLMTM pathogenesis. ..
  15. Kemmerling U, Muñoz P, Müller M, Sanchez G, Aylwin M, Klann E, et al. Calcium release by ryanodine receptors mediates hydrogen peroxide-induced activation of ERK and CREB phosphorylation in N2a cells and hippocampal neurons. Cell Calcium. 2007;41:491-502 pubmed
  16. Tilgen N, Zorzato F, Halliger Keller B, Muntoni F, Sewry C, Palmucci L, et al. Identification of four novel mutations in the C-terminal membrane spanning domain of the ryanodine receptor 1: association with central core disease and alteration of calcium homeostasis. Hum Mol Genet. 2001;10:2879-87 pubmed
    The skeletal muscle ryanodine receptor gene (RYR1; OMIM 180901) on chromosome 19q13.1 encodes the skeletal muscle calcium release channel. To date, more than 25 missense mutations have been identified in RYR1 and are associated with ..
  17. Norman J, Perry S, Reynolds H, Kiebala M, de Mesy Bentley K, Trejo M, et al. HIV-1 Tat activates neuronal ryanodine receptors with rapid induction of the unfolded protein response and mitochondrial hyperpolarization. PLoS ONE. 2008;3:e3731 pubmed publisher
    ..Collectively, these data suggest that abnormal RyR signaling mediates the neuronal UPR with failure of mitochondrial energy metabolism, and is a critical locus for the neuropathogenesis of HIV-1 in the CNS. ..
  18. Jiao G, Hao L, Gao C, Chen L, Sun X, Yang H, et al. Reduced DHPR?1S and RyR1 expression levels are associated with diaphragm contractile dysfunction during sepsis. Muscle Nerve. 2013;48:745-51 pubmed publisher
    ..Dihydropyridine receptors (DHPR?1s and DHPR?1c) and ryanodine receptors (RyR1, RyR2, and RyR3) are essential for excitation-contraction coupling in striated muscles...
  19. Flucher B, Conti A, Takeshima H, Sorrentino V. Type 3 and type 1 ryanodine receptors are localized in triads of the same mammalian skeletal muscle fibers. J Cell Biol. 1999;146:621-30 pubmed
    ..However, in contrast to the skeletal muscle isoform (RyR1), neither the subcellular distribution nor the physiological role of RyR3 are known...
  20. Wang Y, Zheng Y, Mei Q, Wang Q, Collier M, Fleischer S, et al. FKBP12.6 and cADPR regulation of Ca2+ release in smooth muscle cells. Am J Physiol Cell Physiol. 2004;286:C538-46 pubmed
    ..Taken together, these findings point to an important role of the FKBP12.6/RyR2 complex in stochastic (spontaneous) and receptor-mediated Ca2+ release in smooth muscle. ..
  21. Morel J, Dabertrand F, Porte Y, Prevot A, Macrez N. Up-regulation of ryanodine receptor expression increases the calcium-induced calcium release and spontaneous calcium signals in cerebral arteries from hindlimb unloaded rats. Pflugers Arch. 2014;466:1517-28 pubmed
    ..Finally, the expression of the ryanodine receptor subtype 1 (RyR1) was increased in hindlimb unloading conditions...
  22. Samso M, Trujillo R, Gurrola G, Valdivia H, Wagenknecht T. Three-dimensional location of the imperatoxin A binding site on the ryanodine receptor. J Cell Biol. 1999;146:493-9 pubmed
    ..imperatoxin A (IpTx(a)) on the architecture of the calcium release channel/ryanodine receptor from skeletal muscle (RyR1). IpTx(a) is a peptide toxin that binds with high affinity to RyR1 and affects its functioning...
  23. Luo H, Yang H, Jiang R. [Androgen correlates with expressions of ryanodine receptor 1 and voltage-gated calcium channel 1.3 in rat corpus cavernosum smooth muscle]. Zhonghua Nan Ke Xue. 2009;15:895-900 pubmed
    To study the expressions of ryanodine receptor 1 (RyR1) and voltage-gated calcium channel 1.3 (CaV1...
  24. Martin C, Hyvelin J, Chapman K, Marthan R, Ashley R, Savineau J. Pregnant rat myometrial cells show heterogeneous ryanodine- and caffeine-sensitive calcium stores. Am J Physiol. 1999;277:C243-52 pubmed
    ..three ryr mRNAs in the myometrium: ryr3 mRNA is the predominant subtype, with much lower levels of expression for ryr1 and ryr2 mRNAs, suggesting that the ryanodine Ca(2+) release mechanism in rat myometrium is largely encoded by ryr3...
  25. Murayama T, Kurebayashi N, Yamazawa T, Oyamada H, Suzuki J, Kanemaru K, et al. Divergent Activity Profiles of Type 1 Ryanodine Receptor Channels Carrying Malignant Hyperthermia and Central Core Disease Mutations in the Amino-Terminal Region. PLoS ONE. 2015;10:e0130606 pubmed publisher
    The type 1 ryanodine receptor (RyR1) is a Ca2+ release channel in the sarcoplasmic reticulum of skeletal muscle and is mutated in several diseases, including malignant hyperthermia (MH) and central core disease (CCD)...
  26. Obermair G, Kugler G, Baumgartner S, Tuluc P, Grabner M, Flucher B. The Ca2+ channel alpha2delta-1 subunit determines Ca2+ current kinetics in skeletal muscle but not targeting of alpha1S or excitation-contraction coupling. J Biol Chem. 2005;280:2229-37 pubmed
    ..However, this subunit is not essential for targeting of Ca(2+) channels or for their primary physiological role in activating skeletal muscle excitation-contraction coupling. ..
  27. Husain S, Prasad P, Grant W, Kolodecik T, Nathanson M, Gorelick F. The ryanodine receptor mediates early zymogen activation in pancreatitis. Proc Natl Acad Sci U S A. 2005;102:14386-91 pubmed
    ..Inhibition of RYR also inhibited zymogen activation in vivo. We propose that Ca(2+) release from the RYR mediates zymogen activation but not enzyme secretion. The findings imply a role for the RYR in acute pancreatitis. ..
  28. Swift F, Franzini Armstrong C, Øyehaug L, Enger U, Andersson K, Christensen G, et al. Extreme sarcoplasmic reticulum volume loss and compensatory T-tubule remodeling after Serca2 knockout. Proc Natl Acad Sci U S A. 2012;109:3997-4001 pubmed publisher
    ..T-tubule proliferation occurs without loss of the original ordered transverse orientation and thus constitutes the basis for compensation of the declining SR function without structural disarrangement...
  29. Avila G, Lee E, Perez C, Allen P, Dirksen R. FKBP12 binding to RyR1 modulates excitation-contraction coupling in mouse skeletal myotubes. J Biol Chem. 2003;278:22600-8 pubmed
    The skeletal muscle sarcoplasmic reticulum (SR) Ca2+ release channel or ryanodine receptor (RyR1) binds four molecules of FKBP12, and the interaction of FKBP12 with RyR1 regulates both unitary and coupled gating of the channel...
  30. Futatsugi A, Kuwajima G, Mikoshiba K. Tissue-specific and developmentally regulated alternative splicing in mouse skeletal muscle ryanodine receptor mRNA. Biochem J. 1995;305 ( Pt 2):373-8 pubmed
    ..By PCR analysis, we identified two alternatively spliced regions in mRNA of the mouse skeletal muscle ryanodine receptor (sRyR)...
  31. Gregg R, Messing A, Strube C, Beurg M, Moss R, Behan M, et al. Absence of the beta subunit (cchb1) of the skeletal muscle dihydropyridine receptor alters expression of the alpha 1 subunit and eliminates excitation-contraction coupling. Proc Natl Acad Sci U S A. 1996;93:13961-6 pubmed
  32. Kramerova I, Kudryashova E, Wu B, Ottenheijm C, Granzier H, Spencer M. Novel role of calpain-3 in the triad-associated protein complex regulating calcium release in skeletal muscle. Hum Mol Genet. 2008;17:3271-80 pubmed publisher
    ..Together, these data suggest that CAPN3 is necessary for the structural integrity of the triad-associated protein complex and that impairment of calcium transport is a phenotypic feature of CAPN3-deficient muscle. ..
  33. Lacava C, Michalek Sauberer A, Kraft B, Sgaragli G, Sipos E, Höller C, et al. 3,5-Di-t-butyl catechol is a potent human ryanodine receptor 1 activator, not suitable for the diagnosis of malignant hyperthermia susceptibility. Pharmacol Res. 2012;66:80-7 pubmed publisher
    ..However, the sensitivity of MHS or MHEH muscles to DTCAT was similar to that of MHN muscles. In conclusion, DTCAT is not suitable for the diagnosis of MH susceptibility due to its failure to discriminate between MHN and MHS muscles. ..
  34. Jurkovicova D, Sedlakova B, Lacinova L, Kopacek J, Sulova Z, Sedlak J, et al. Hypoxia differently modulates gene expression of inositol 1,4,5-trisphosphate receptors in mouse kidney and HEK 293 cell line. Ann N Y Acad Sci. 2008;1148:421-7 pubmed publisher
    ..RyR2 mRNA in HEK 293 cells was not significantly changed, but RyR1 gene expression was significantly increased by hypoxia...
  35. Bastide B, Snoeckx K, Mounier Y. ADP-ribose stimulates the calcium release channel RyR1 in skeletal muscle of rat. Biochem Biophys Res Commun. 2002;296:1267-71 pubmed
    ..We studied the effect of ADPr on the Ca(2+) release channel of skeletal muscle RyR1 after incorporation of microsomes isolated from fast muscles of rat in planar lipid bilayers...
  36. Bellinger A, Reiken S, Carlson C, Mongillo M, Liu X, Rothman L, et al. Hypernitrosylated ryanodine receptor calcium release channels are leaky in dystrophic muscle. Nat Med. 2009;15:325-30 pubmed publisher
    ..We have identified a structural and functional defect in the ryanodine receptor (RyR1), a sarcoplasmic reticulum Ca2+ release channel, in the mdx mouse model of muscular dystrophy that contributes to ..
  37. Luo Y, Jiang R. [Expressions of Cav1.3 and RyR1 in the corpus cavernosum of the penis in aged rats]. Zhonghua Nan Ke Xue. 2009;15:985-9 pubmed
    ..3 and RyR1 in the corpus cavernosum of the penis by RT-PCR and immunohistochemistry...
  38. Shtifman A, Paolini C, Lopez J, Allen P, Protasi F. Ca2+ influx through alpha1S DHPR may play a role in regulating Ca2+ release from RyR1 in skeletal muscle. Am J Physiol Cell Physiol. 2004;286:C73-8 pubmed
    ..showed expression of both alpha1S dihydropyridine receptors (DHPRs) and ryanodine-sensitive Ca2+-release channel 1 (RyR1), the two key proteins in skeletal excitation-contraction (E-C) coupling...
  39. Lynn S, Morgan J, Lamb H, Meissner G, Gillespie J. Isolation and partial cloning of ryanodine-sensitive Ca2+ release channel protein isoforms from human myometrial smooth muscle. FEBS Lett. 1995;372:6-12 pubmed
    ..SDS-PAGE of the sucrose gradient fractions indicated the specific comigration of a polypeptide with a molecular mass of approximately 544 kDa with the ryanodine binding activity. ..
  40. Tanaka H, Furuya T, Kameda N, Kobayashi T, Mizusawa H. Triad proteins and intracellular Ca2+ transients during development of human skeletal muscle cells in aneural and innervated cultures. J Muscle Res Cell Motil. 2000;21:507-26 pubmed
  41. Takeshima H, Yamazawa T, Ikemoto T, Takekura H, Nishi M, Noda T, et al. Ca(2+)-induced Ca2+ release in myocytes from dyspedic mice lacking the type-1 ryanodine receptor. EMBO J. 1995;14:2999-3006 pubmed
    ..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. Clark J, Kinnear N, Kalujnaia S, Cramb G, Fleischer S, Jeyakumar L, et al. Identification of functionally segregated sarcoplasmic reticulum calcium stores in pulmonary arterial smooth muscle. J Biol Chem. 2010;285:13542-9 pubmed publisher
    ..arterial smooth muscle cells revealed striking differences in the spatial distribution of SERCA2a and SERCA2b and RyR1, RyR2, and RyR3, respectively. Almost all SERCA2a and RyR3 labeling was restricted to a region within 1...