calsequestrin

Summary

Summary: Acidic protein found in SARCOPLASMIC RETICULUM that binds calcium to the extent of 700-900 nmoles/mg. It plays the role of sequestering calcium transported to the interior of the intracellular vesicle.

Top Publications

  1. Perrino C, Naga Prasad S, Patel M, Wolf M, Rockman H. Targeted inhibition of beta-adrenergic receptor kinase-1-associated phosphoinositide-3 kinase activity preserves beta-adrenergic receptor signaling and prolongs survival in heart failure induced by calsequestrin overexpression. J Am Coll Cardiol. 2005;45:1862-70 pubmed
    ..Desensitization and down-regulation of beta-adrenergic receptors (betaARs) are prominent features of heart failure largely mediated by increased levels of betaAR kinase-1 (betaARK1)...
  2. Sato Y, Kiriazis H, Yatani A, Schmidt A, Hahn H, Ferguson D, et al. Rescue of contractile parameters and myocyte hypertrophy in calsequestrin overexpressing myocardium by phospholamban ablation. J Biol Chem. 2001;276:9392-9 pubmed
    Cardiac-specific overexpression of murine cardiac calsequestrin results in depressed cardiac contractile parameters, low Ca(2+)-induced Ca(2+) release from sarcoplasmic reticulum (SR) and cardiac hypertrophy in transgenic mice...
  3. Kalyanasundaram A, Viatchenko Karpinski S, Belevych A, Lacombe V, Hwang H, Knollmann B, et al. Functional consequences of stably expressing a mutant calsequestrin (CASQ2D307H) in the CASQ2 null background. Am J Physiol Heart Circ Physiol. 2012;302:H253-61 pubmed publisher
    The role of calsequestrin (CASQ2) in cardiac sarcoplasmic reticulum (SR) calcium (Ca(2+)) transport has gained significant attention since point mutations in CASQ2 were reported to cause ventricular arrhythmia...
  4. Denegri M, Avelino Cruz J, Boncompagni S, De Simone S, Auricchio A, Villani L, et al. Viral gene transfer rescues arrhythmogenic phenotype and ultrastructural abnormalities in adult calsequestrin-null mice with inherited arrhythmias. Circ Res. 2012;110:663-8 pubmed publisher
    ..is associated with mutations in the genes encoding for the cardiac ryanodine receptor (RyR2) and cardiac calsequestrin (CASQ2)...
  5. Liu N, Rizzi N, Boveri L, Priori S. Ryanodine receptor and calsequestrin in arrhythmogenesis: what we have learnt from genetic diseases and transgenic mice. J Mol Cell Cardiol. 2009;46:149-59 pubmed publisher
    ..CPVT has been linked to mutations in genes encoding the cardiac ryanodine receptor (RyR2) and cardiac calsequestrin (CASQ2): two fundamental proteins involved in regulation of intracellular Ca(2+) in cardiac myocytes...
  6. Faggioni M, Kryshtal D, Knollmann B. Calsequestrin mutations and catecholaminergic polymorphic ventricular tachycardia. Pediatr Cardiol. 2012;33:959-67 pubmed publisher
    Cardiac calsequestrin (Casq2) is the major Ca2+ binding protein in the sarcoplasmic reticulum, which is the principle Ca2+ storage organelle of cardiac muscle...
  7. Rizzi N, Liu N, Napolitano C, Nori A, Turcato F, Colombi B, et al. Unexpected structural and functional consequences of the R33Q homozygous mutation in cardiac calsequestrin: a complex arrhythmogenic cascade in a knock in mouse model. Circ Res. 2008;103:298-306 pubmed publisher
    ..The recessive form of CPVT is associated with mutation in the cardiac calsequestrin gene (CASQ2)...
  8. Knoll A, Stratil A, Reiner G, Peelman L, Van Poucke M, Geldermann H. Linkage and radiation hybrid mapping of the porcine calsequestrin 1 (CASQ1 ) gene to chromosome 4q. Anim Genet. 2002;33:390-2 pubmed
  9. Lee K, Maeng J, Choi J, Lee Y, Hwang C, Park S, et al. Role of Junctin protein interactions in cellular dynamics of calsequestrin polymer upon calcium perturbation. J Biol Chem. 2012;287:1679-87 pubmed publisher
    b>Calsequestrin (CSQ), the major intrasarcoplasmic reticulum calcium storage protein, undergoes dynamic polymerization and depolymerization in a Ca(2+)-dependent manner...

More Information

Publications74

  1. Kobayashi Y, Alseikhan B, Jones L. Localization and characterization of the calsequestrin-binding domain of triadin 1. Evidence for a charged beta-strand in mediating the protein-protein interaction. J Biol Chem. 2000;275:17639-46 pubmed
    ..protein of the junctional sarcoplasmic reticulum that binds to the high capacity Ca(2+)-binding protein calsequestrin and anchors it to the ryanodine receptor...
  2. Nguyen B, Gopinath B, Tani J, Wescombe L, Wall J. Peripheral blood T lymphocyte sensitisation against calsequestrin and flavoprotein in patients with Graves' ophthalmopathy. Autoimmunity. 2008;41:372-6 pubmed publisher
    ..Therefore, we tested for T lymphocyte sensitisation to three eye muscle antigens namely, calsequestrin, G2s and flavoprotein (Fp), in patients with Graves' ophthalmopathy (GO), Graves' hyperthyroidism (GH) without ..
  3. Qin J, Valle G, Nani A, Nori A, Rizzi N, Priori S, et al. Luminal Ca2+ regulation of single cardiac ryanodine receptors: insights provided by calsequestrin and its mutants. J Gen Physiol. 2008;131:325-34 pubmed publisher
    ..5 mm. ..
  4. Korajoki H, Vornanen M. Expression of calsequestrin in atrial and ventricular muscle of thermally acclimated rainbow trout. J Exp Biol. 2009;212:3403-14 pubmed publisher
    b>Calsequestrin (CASQ) is the main Ca(2+) binding protein within the sarcoplasmic reticulum (SR) of the vertebrate heart...
  5. Terentyev D, Viatchenko Karpinski S, Vedamoorthyrao S, Oduru S, Gyorke I, Williams S, et al. Protein protein interactions between triadin and calsequestrin are involved in modulation of sarcoplasmic reticulum calcium release in cardiac myocytes. J Physiol. 2007;583:71-80 pubmed
    ..intracellular Ca2+ release is controlled by a number of proteins including the ryanodine receptor (RyR2), calsequestrin (CASQ2), triadin-1 (Trd) and junctin (Jn) which form a complex in the junctional sarcoplasmic reticulum (SR) ..
  6. Alcalai R, Wakimoto H, Arad M, Planer D, Konno T, Wang L, et al. Prevention of ventricular arrhythmia and calcium dysregulation in a catecholaminergic polymorphic ventricular tachycardia mouse model carrying calsequestrin-2 mutation. J Cardiovasc Electrophysiol. 2011;22:316-24 pubmed publisher
    ..caused by mutations in genes encoding the calcium-regulation proteins cardiac ryanodine receptor (RyR2) or calsequestrin-2 (CASQ2)...
  7. Protasi F, Paolini C, Canato M, Reggiani C, Quarta M. Lessons from calsequestrin-1 ablation in vivo: much more than a Ca(2+) buffer after all. J Muscle Res Cell Motil. 2011;32:257-70 pubmed publisher
    b>Calsequestrin type-1 (CASQ1), the main sarcoplasmic reticulum (SR) Ca(2+) binding protein, plays a dual role in skeletal fibers: a) it provides a large pool of rapidly-releasable Ca(2+) during excitation-contraction (EC) coupling; and b) ..
  8. Cho J, Oh Y, Park K, Yu J, Choi K, Shin J, et al. Calsequestrin, a calcium sequestering protein localized at the sarcoplasmic reticulum, is not essential for body-wall muscle function in Caenorhabditis elegans. J Cell Sci. 2000;113 ( Pt 22):3947-58 pubmed
    b>Calsequestrin is the major calcium-binding protein of cardiac and skeletal muscles whose function is to sequester Ca(2+ )in the lumen of the sarcoplasmic reticulum (SR)...
  9. Jacob S, Sleiman N, Kern S, Jones L, Sala Mercado J, McFarland T, et al. Altered calsequestrin glycan processing is common to diverse models of canine heart failure. Mol Cell Biochem. 2013;377:11-21 pubmed publisher
    b>Calsequestrin-2 (CSQ2) is a resident glycoprotein of junctional sarcoplasmic reticulum that functions in the regulation of SR Ca(2+) release...
  10. Beard N, Casarotto M, Wei L, Varsanyi M, Laver D, Dulhunty A. Regulation of ryanodine receptors by calsequestrin: effect of high luminal Ca2+ and phosphorylation. Biophys J. 2005;88:3444-54 pubmed
    b>Calsequestrin, the major calcium sequestering protein in the sarcoplasmic reticulum of muscle, forms a quaternary complex with the ryanodine receptor calcium release channel and the intrinsic membrane proteins triadin and junctin...
  11. Zarain Herzberg A, Estrada Avilés R, Fragoso Medina J. Regulation of sarco(endo)plasmic reticulum Ca2+-ATPase and calsequestrin gene expression in the heart. Can J Physiol Pharmacol. 2012;90:1017-28 pubmed publisher
    ..The SR Ca(2+)-ATPase (SERCA2) transports Ca(2+) inside the SR lumen during relaxation of the cardiac myocyte. Calsequestrin (Casq2) is the main protein in the SR lumen, functioning as a Ca(2+) buffer and participating in Ca(2+) ..
  12. Valle G, Galla D, Nori A, Priori S, Gyorke S, De Filippis V, et al. Catecholaminergic polymorphic ventricular tachycardia-related mutations R33Q and L167H alter calcium sensitivity of human cardiac calsequestrin. Biochem J. 2008;413:291-303 pubmed publisher
    Two missense mutations, R33Q and L167H, of hCASQ2 (human cardiac calsequestrin), a protein segregated to the lumen of the sarcoplasmic reticulum, are linked to the autosomal recessive form of CPVT (catecholaminergic polymorphic ..
  13. Fliegel L, Ohnishi M, Carpenter M, Khanna V, Reithmeier R, MacLennan D. Amino acid sequence of rabbit fast-twitch skeletal muscle calsequestrin deduced from cDNA and peptide sequencing. Proc Natl Acad Sci U S A. 1987;84:1167-71 pubmed
    Partial amino acid sequence analysis of rabbit fast-twitch skeletal muscle calsequestrin permitted the construction of synthetic oligonucleotides that were used as both primers and probes for the synthesis and isolation of cDNAs encoding ..
  14. Sanchez E, Lewis K, Danna B, Kang C. High-capacity Ca2+ binding of human skeletal calsequestrin. J Biol Chem. 2012;287:11592-601 pubmed publisher
    b>Calsequestrin, the major calcium storage protein in both cardiac and skeletal muscle, binds large amounts of Ca(2+) in the sarcoplasmic reticulum and releases them during muscle contraction...
  15. Guo W, Jorgensen A, Jones L, Campbell K. Biochemical characterization and molecular cloning of cardiac triadin. J Biol Chem. 1996;271:458-65 pubmed
    ..that the conserved region of the luminal domain of triadin is able to bind both the ryanodine receptor and calsequestrin in cardiac muscle...
  16. Viatchenko Karpinski S, Terentyev D, Gyorke I, Terentyeva R, Volpe P, Priori S, et al. Abnormal calcium signaling and sudden cardiac death associated with mutation of calsequestrin. Circ Res. 2004;94:471-7 pubmed
    Mutations in human cardiac calsequestrin (CASQ2), a high-capacity calcium-binding protein located in the sarcoplasmic reticulum (SR), have recently been linked to effort-induced ventricular arrhythmia and sudden death (catecholaminergic ..
  17. Wei L, Gallant E, Dulhunty A, Beard N. Junctin and triadin each activate skeletal ryanodine receptors but junctin alone mediates functional interactions with calsequestrin. Int J Biochem Cell Biol. 2009;41:2214-24 pubmed publisher
    ..triadin and junctin and their ability to mediate functional interactions between the Ca(2+) binding protein calsequestrin and the type 1 ryanodine receptor in the lumen of the sarcoplasmic reticulum...
  18. Ohkura M, Furukawa K, Fujimori H, Kuruma A, Kawano S, Hiraoka M, et al. Dual regulation of the skeletal muscle ryanodine receptor by triadin and calsequestrin. Biochemistry. 1998;37:12987-93 pubmed
    Triadin, a calsequestrin-anchoring transmembrane protein of the sarcoplasmic reticulum (SR), was successfully purified from the heavy fraction of SR (HSR) of rabbit skeletal muscle with an anti-triadin immunoaffinity column...
  19. Domeier T, Blatter L, Zima A. Alteration of sarcoplasmic reticulum Ca2+ release termination by ryanodine receptor sensitization and in heart failure. J Physiol. 2009;587:5197-209 pubmed publisher
    ..Collectively, these data suggest that altering the termination level for local Ca(2+) release may represent a novel mechanism to increase SR Ca(2+) release and contractility during ECC. ..
  20. Sato Y, Ferguson D, Sako H, Dorn G, Kadambi V, Yatani A, et al. Cardiac-specific overexpression of mouse cardiac calsequestrin is associated with depressed cardiovascular function and hypertrophy in transgenic mice. J Biol Chem. 1998;273:28470-7 pubmed
    b>Calsequestrin is a high capacity Ca2+-binding protein in the sarcoplasmic reticulum (SR) lumen...
  21. Royer L, Sztretye M, Manno C, Pouvreau S, Zhou J, Knollmann B, et al. Paradoxical buffering of calcium by calsequestrin demonstrated for the calcium store of skeletal muscle. J Gen Physiol. 2010;136:325-38 pubmed publisher
    ..requires a Ca(2+) reservoir of large capacity inside the sarcoplasmic reticulum (SR), presumably the protein calsequestrin. The buffering power of calsequestrin in vitro has a paradoxical dependence on [Ca(2+)] that should be ..
  22. Priori S, Chen S. Inherited dysfunction of sarcoplasmic reticulum Ca2+ handling and arrhythmogenesis. Circ Res. 2011;108:871-83 pubmed publisher
    ..In 2001, the ryanodine receptor was identified as the gene that is linked to CPVT; shortly thereafter, cardiac calsequestrin was implicated in the recessive form of the same disease...
  23. Ramay H, Liu O, Sobie E. Recovery of cardiac calcium release is controlled by sarcoplasmic reticulum refilling and ryanodine receptor sensitivity. Cardiovasc Res. 2011;91:598-605 pubmed publisher
  24. Bal N, Sharon A, Gupta S, Jena N, Shaikh S, Gyorke S, et al. The catecholaminergic polymorphic ventricular tachycardia mutation R33Q disrupts the N-terminal structural motif that regulates reversible calsequestrin polymerization. J Biol Chem. 2010;285:17188-96 pubmed publisher
    b>Calsequestrin undergoes dynamic polymerization with increasing calcium concentration by front-to-front dimerization and back-to-back packing, forming wire-shaped structures...
  25. Song L, Alcalai R, Arad M, Wolf C, Toka O, Conner D, et al. Calsequestrin 2 (CASQ2) mutations increase expression of calreticulin and ryanodine receptors, causing catecholaminergic polymorphic ventricular tachycardia. J Clin Invest. 2007;117:1814-23 pubmed
    ..ventricular tachycardia (CPVT) is a familial disorder caused by cardiac ryanodine receptor type 2 (RyR2) or calsequestrin 2 (CASQ2) gene mutations...
  26. Seehase M, Quentin T, Wiludda E, Hellige G, Paul T, Schiffmann H. Gene expression of the Na-Ca2+ exchanger, SERCA2a and calsequestrin after myocardial ischemia in the neonatal rabbit heart. Biol Neonate. 2006;90:174-84 pubmed
    ..expression of the sodium-calcium exchanger (NCX), the sarco-endoplasmatic reticulum Ca2+-ATPase 2a (SERCA) and calsequestrin (CSQ) were measured using quantitative real-time PCR and Western blotting...
  27. Beard N, Sakowska M, Dulhunty A, Laver D. Calsequestrin is an inhibitor of skeletal muscle ryanodine receptor calcium release channels. Biophys J. 2002;82:310-20 pubmed
    We provide novel evidence that the sarcoplasmic reticulum calcium binding protein, calsequestrin, inhibits native ryanodine receptor calcium release channel activity...
  28. Wang Y, Xu L, Duan H, Pasek D, Eu J, Meissner G. Knocking down type 2 but not type 1 calsequestrin reduces calcium sequestration and release in C2C12 skeletal muscle myotubes. J Biol Chem. 2006;281:15572-81 pubmed
    ..Taken together, our data suggest that knocking down CSQ2, but not CSQ1, leads to reduced Ca2+ storage and release in C2C12 myotubes. ..
  29. Kirchhefer U, Hanske G, Jones L, Justus I, Kaestner L, Lipp P, et al. Overexpression of junctin causes adaptive changes in cardiac myocyte Ca(2+) signaling. Cell Calcium. 2006;39:131-42 pubmed
    In cardiac muscle, junctin forms a quaternary protein complex with the ryanodine receptor (RyR), calsequestrin, and triadin 1 at the luminal face of the junctional sarcoplasmic reticulum (jSR)...
  30. Hidalgo C, Donoso P, Rodriguez P. Protons induce calsequestrin conformational changes. Biophys J. 1996;71:2130-7 pubmed
    b>Calsequestrin, a high-capacity, intermediate-affinity, calcium-binding protein present in the lumen of sarcoplasmic reticulum, undergoes extensive calcium-induced conformational changes at neutral pH that cause distinct intrinsic ..
  31. Allen B, Katz S. Calreticulin and calsequestrin are differentially distributed in canine heart. J Mol Cell Cardiol. 2000;32:2379-84 pubmed
    ..In the SR, Ca(2+)is stored in a complex with Ca(2+)-binding proteins such as calsequestrin. In the present study, the distribution of the high-affinity calcium-binding protein, calreticulin, in canine ..
  32. Dinchuk J, Henderson N, Burn T, Huber R, Ho S, Link J, et al. Aspartyl beta -hydroxylase (Asph) and an evolutionarily conserved isoform of Asph missing the catalytic domain share exons with junctin. J Biol Chem. 2000;275:39543-54 pubmed
    ..The gene for mouse junctin, a calsequestrin-binding protein, was cloned and characterized and shown to be encoded from the same locus...
  33. Suzuki Y, Ikeda T, Shi S, Kitta K, Kobayashi Y, Morad M, et al. Regulation of GATA-4 and AP-1 in transgenic mice overexpressing cardiac calsequestrin. Cell Calcium. 1999;25:401-7 pubmed
    Transgenic mouse hearts overexpressing the Ca(2+)-binding protein calsequestrin (CSQ) have an accompanying 10-fold increase in the sarcoplasmic reticulum (SR) Ca2+ load, however, exhibits slow and small Ca(2+)-induced Ca2+ release...
  34. Laitinen P, Swan H, Kontula K. Molecular genetics of exercise-induced polymorphic ventricular tachycardia: identification of three novel cardiac ryanodine receptor mutations and two common calsequestrin 2 amino-acid polymorphisms. Eur J Hum Genet. 2003;11:888-91 pubmed
    Mutations of two myocardial calcium signaling molecules, ryanodine receptor 2 (RYR2) and calsequestrin 2 (CASQ2), may cause catecholaminergic polymorphic ventricular tachycardia (CPVT), a severe inherited arrhythmic disease manifesting ..
  35. Wang Y, Li X, Duan H, Fulton T, Eu J, Meissner G. Altered stored calcium release in skeletal myotubes deficient of triadin and junctin. Cell Calcium. 2009;45:29-37 pubmed publisher
    ..that in junctin knockdown myotubes at least two sarcoplasmic reticulum proteins (RyR1 and skeletal muscle calsequestrin) were down-regulated while these proteins' mRNA expression was not affected...
  36. Shin D, Ma J, Kim D. The asp-rich region at the carboxyl-terminus of calsequestrin binds to Ca(2+) and interacts with triadin. FEBS Lett. 2000;486:178-82 pubmed
    b>Calsequestrin (CSQ) is a high capacity Ca(2+) binding protein in the junctional sarcoplasmic reticulum of striated muscles, and has been shown to regulate the ryanodine receptor (RyR) through triadin and junctin...
  37. Ihara Y, Suzuki Y, Kitta K, Jones L, Ikeda T. Modulation of gene expression in transgenic mouse hearts overexpressing calsequestrin. Cell Calcium. 2002;32:21-9 pubmed
    b>Calsequestrin (CSQ) is the major Ca2+ binding protein of the cardiac sarcoplasmic reticulum (SR)...
  38. Park H, Wu S, Dunker A, Kang C. Polymerization of calsequestrin. Implications for Ca2+ regulation. J Biol Chem. 2003;278:16176-82 pubmed
    Two distinct dimerization contacts in calsequestrin crystals suggested a mechanism for Ca(2+) regulation resulting from the occurrence of coupled Ca(2+) binding and protein polymerization...
  39. Takei K, Stukenbrok H, Metcalf A, Mignery G, Sudhof T, Volpe P, et al. Ca2+ stores in Purkinje neurons: endoplasmic reticulum subcompartments demonstrated by the heterogeneous distribution of the InsP3 receptor, Ca(2+)-ATPase, and calsequestrin. J Neurosci. 1992;12:489-505 pubmed
    ..involved in Ca2+ storage and release: the inositol 1,4,5-trisphosphate (InsP3) receptor, Ca(2+)-ATPase, and calsequestrin. Ca(2+)-ATPase and the InsP3 receptor have a widespread, although not identical, distribution throughout the ..
  40. Terentyev D, Viatchenko Karpinski S, Gyorke I, Volpe P, Williams S, Gyorke S. Calsequestrin determines the functional size and stability of cardiac intracellular calcium stores: Mechanism for hereditary arrhythmia. Proc Natl Acad Sci U S A. 2003;100:11759-64 pubmed
    b>Calsequestrin is a high-capacity Ca-binding protein expressed inside the sarcoplasmic reticulum (SR), an intracellular Ca release and storage organelle in muscle...
  41. Sztretye M, Yi J, Figueroa L, Zhou J, Royer L, Rios E. D4cpv-calsequestrin: a sensitive ratiometric biosensor accurately targeted to the calcium store of skeletal muscle. J Gen Physiol. 2011;138:211-29 pubmed publisher
    ..range, which may be degraded further by imperfect targeting and interference from endogenous ligands of calsequestrin. We describe a novel tool that uses the cameleon D4cpv, which has a greater dynamic range and lower ..
  42. Beard N, Laver D, Dulhunty A. Calsequestrin and the calcium release channel of skeletal and cardiac muscle. Prog Biophys Mol Biol. 2004;85:33-69 pubmed
    b>Calsequestrin is by far the most abundant Ca(2+)-binding protein in the sarcoplasmic reticulum (SR) of skeletal and cardiac muscle...
  43. Kubalova Z, Gyorke I, Terentyeva R, Viatchenko Karpinski S, Terentyev D, Williams S, et al. Modulation of cytosolic and intra-sarcoplasmic reticulum calcium waves by calsequestrin in rat cardiac myocytes. J Physiol. 2004;561:515-24 pubmed
    ..ventricular tachycardia (CPVT), certain cases of which are associated with mutations in the cardiac calsequestrin gene (CASQ2)...
  44. Guo W, Campbell K. Association of triadin with the ryanodine receptor and calsequestrin in the lumen of the sarcoplasmic reticulum. J Biol Chem. 1995;270:9027-30 pubmed
    ..that triadin binds specifically to the ryanodine receptor/Ca2+ release channel and the Ca(2+)-binding protein calsequestrin from CHAPS (3-[(3-cholamidopropyl)dimethylammonio]-1-propanesulfonic acid)-solubilized skeletal muscle ..
  45. Pape P, Fenelon K, Lamboley C, Stachura D. Role of calsequestrin evaluated from changes in free and total calcium concentrations in the sarcoplasmic reticulum of frog cut skeletal muscle fibres. J Physiol. 2007;581:319-67 pubmed
    b>Calsequestrin is a large-capacity Ca-binding protein located in the terminal cisternae of sarcoplasmic reticulum (SR) suggesting a role as a buffer of the concentration of free Ca in the SR ([Ca2+](SR)) serving to maintain the driving ..
  46. Slupsky J, Ohnishi M, Carpenter M, Reithmeier R. Characterization of cardiac calsequestrin. Biochemistry. 1987;26:6539-44 pubmed
    b>Calsequestrin, a calcium-binding protein found in the sarcoplasmic reticulum of muscle cells, was purified from rabbit and canine cardiac and skeletal muscle tissue...
  47. Choi E, Clegg D. Identification and developmental expression of a chicken calsequestrin homolog. Dev Biol. 1990;142:169-77 pubmed
    b>Calsequestrin (CAL) is a calcium-binding protein whose primary function is thought to involve sequestration of calcium in the muscle sarcoplasmic reticulum (SR)...
  48. Royer L, Rios E. Deconstructing calsequestrin. Complex buffering in the calcium store of skeletal muscle. J Physiol. 2009;587:3101-11 pubmed publisher
    Since its discovery in 1971, calsequestrin has been recognized as the main Ca(2+) binding protein inside the sarcoplasmic reticulum (SR), the organelle that stores and upon demand mobilizes Ca(2+) for contractile activation of muscle...
  49. Szegedi C, Sarkozi S, Herzog A, Jona I, Varsanyi M. Calsequestrin: more than 'only' a luminal Ca2+ buffer inside the sarcoplasmic reticulum. Biochem J. 1999;337 ( Pt 1):19-22 pubmed
    ..for maintaining the free [Ca2+] at 10(-3)-10(-4) M in the SR lumen; in skeletal-muscle SR, it is mainly calsequestrin. Here we show that, depending on its phosphorylation state, calsequestrin selectively controls the RyR channel ..
  50. Wang S, Trumble W, Liao H, Wesson C, Dunker A, Kang C. Crystal structure of calsequestrin from rabbit skeletal muscle sarcoplasmic reticulum. Nat Struct Biol. 1998;5:476-83 pubmed
    b>Calsequestrin, the major Ca2+ storage protein of muscle, coordinately binds and releases 40-50 Ca2+ ions per molecule for each contraction-relaxation cycle by an uncertain mechanism...
  51. Infante C, Ponce M, Manchado M. Duplication of calsequestrin genes in teleosts: molecular characterization in the Senegalese sole (Solea senegalensis). Comp Biochem Physiol B Biochem Mol Biol. 2011;158:304-14 pubmed publisher
    b>Calsequestrin is a moderate-affinity, high-capacity Ca(2+) binding protein in the sarcoplasmic reticulum of skeletal and cardiac muscle that seems to act as an intralumenal Ca(2+) buffer...
  52. Mulvey C, Ohlendieck K. Use of continuous-elution gel electrophoresis as a preparative tool for blot overlay analysis. Anal Biochem. 2003;319:122-30 pubmed
    ..Since the supramolecular structure of calsequestrin has recently been determined, this terminal cisternae protein was isolated as a model protein for studying ..
  53. Wei L, Hanna A, Beard N, Dulhunty A. Unique isoform-specific properties of calsequestrin in the heart and skeletal muscle. Cell Calcium. 2009;45:474-84 pubmed publisher
    ..RyR2) calcium release channel and the calcium buffering protein in the sarcoplasmic reticulum, cardiac calsequestrin (CSQ2)...
  54. Kiarash A, Kelly C, Phinney B, Valdivia H, Abrams J, Cala S. Defective glycosylation of calsequestrin in heart failure. Cardiovasc Res. 2004;63:264-72 pubmed
    ..Measures of calsequestrin (CSQ), however, a critical protein component of the Ca2+ release complex in junctional sarcoplasmic reticulum, ..
  55. Doran P, Dowling P, Lohan J, McDonnell K, Poetsch S, Ohlendieck K. Subproteomics analysis of Ca+-binding proteins demonstrates decreased calsequestrin expression in dystrophic mouse skeletal muscle. Eur J Biochem. 2004;271:3943-52 pubmed
    ..sarcoplasmic reticulum, surprisingly no changes in the abundance of the main luminal Ca(2+) reservoir protein calsequestrin have been observed in microsomal preparations...
  56. Shen X, Franzini Armstrong C, Lopez J, Jones L, Kobayashi Y, Wang Y, et al. Triadins modulate intracellular Ca(2+) homeostasis but are not essential for excitation-contraction coupling in skeletal muscle. J Biol Chem. 2007;282:37864-74 pubmed
    ..that the absence of triadin expression was associated with down-regulation of Junctophilin-1, junctin, and calsequestrin but resulted in no obvious contractile dysfunction...
  57. Chopra N, Kannankeril P, Yang T, Hlaing T, Holinstat I, Ettensohn K, et al. Modest reductions of cardiac calsequestrin increase sarcoplasmic reticulum Ca2+ leak independent of luminal Ca2+ and trigger ventricular arrhythmias in mice. Circ Res. 2007;101:617-26 pubmed
    Cardiac calsequestrin-null mice (Casq2-/-) display catecholaminergic ventricular tachycardia akin to humans with CASQ2 mutations...
  58. Shen J, Cronin C, Sonin D, Joshi B, Gongora Nieto M, Harrison D, et al. P2X purinergic receptor-mediated ionic current in cardiac myocytes of calsequestrin model of cardiomyopathy: implications for the treatment of heart failure. Am J Physiol Heart Circ Physiol. 2007;292:H1077-84 pubmed
    ..was to characterize the P2X receptor-mediated ionic current and determine its role in heart failure using the calsequestrin (CSQ) model of cardiomyopathy...
  59. di Barletta M, Viatchenko Karpinski S, Nori A, Memmi M, Terentyev D, Turcato F, et al. Clinical phenotype and functional characterization of CASQ2 mutations associated with catecholaminergic polymorphic ventricular tachycardia. Circulation. 2006;114:1012-9 pubmed
    Four distinct mutations in the human cardiac calsequestrin gene (CASQ2) have been linked to catecholaminergic polymorphic ventricular tachycardia (CPVT)...
  60. Zhang L, Franzini Armstrong C, Ramesh V, Jones L. Structural alterations in cardiac calcium release units resulting from overexpression of junctin. J Mol Cell Cardiol. 2001;33:233-47 pubmed
    Junctin is a 26 kDa membrane protein that binds to calsequestrin, triadin, and ryanodine receptors (RyRs) within the junctional sarcoplasmic reticulum of calcium release units...
  61. Liu J, Lieu D, Siu C, Fu J, Tse H, Li R. Facilitated maturation of Ca2+ handling properties of human embryonic stem cell-derived cardiomyocytes by calsequestrin expression. Am J Physiol Cell Physiol. 2009;297:C152-9 pubmed publisher
    ..In particular, calsequestrin (CSQ), the most abundant, high-capacity but low-affinity, Ca(2+)-binding protein in the SR that is anchored to ..
  62. Park H, Park I, Kim E, Youn B, Fields K, Dunker A, et al. Comparing skeletal and cardiac calsequestrin structures and their calcium binding: a proposed mechanism for coupled calcium binding and protein polymerization. J Biol Chem. 2004;279:18026-33 pubmed
    b>Calsequestrin, the major calcium storage protein of both cardiac and skeletal muscle, binds and releases large numbers of Ca(2+) ions for each contraction and relaxation cycle...
  63. Ikemoto N, Ronjat M, Meszaros L, Koshita M. Postulated role of calsequestrin in the regulation of calcium release from sarcoplasmic reticulum. Biochemistry. 1989;28:6764-71 pubmed
    ..Since most of the intravesicular calcium appears to be bound to calsequestrin both under passive and under active loading conditions, these results suggest that the kinetic properties of ..