Gene Symbol: Casq1
Description: calsequestrin 1
Alias: CSQ, CSQ-1, CSQ1, sCSQ, calsequestrin-1, calmitine, calsequestrin, skeletal muscle isoform
Species: mouse
Products:     Casq1

Top Publications

  1. 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
    We examined the roles of type 1 and type 2 calsequestrins (CSQ1 and CSQ2) in stored Ca2+ release of C2C12 skeletal muscle myotubes...
  2. Shin D, Pan Z, Kim E, Lee J, Bhat M, Parness J, et al. A retrograde signal from calsequestrin for the regulation of store-operated Ca2+ entry in skeletal muscle. J Biol Chem. 2003;278:3286-92 pubmed
    Calsequestrin (CSQ) is a high capacity Ca(2+)-binding protein present in the lumen of sarcoplasmic reticulum (SR) in striated muscle cells and has been shown to regulate the ryanodine receptor Ca(2+) release channel activity through ..
  3. Dainese M, Quarta M, Lyfenko A, Paolini C, Canato M, Reggiani C, et al. Anesthetic- and heat-induced sudden death in calsequestrin-1-knockout mice. FASEB J. 2009;23:1710-20 pubmed publisher
    Calsequestrin-1 (CASQ1) is a moderate-affinity, high-capacity Ca(2+)-binding protein in the sarcoplasmic reticulum (SR) terminal cisternae of skeletal muscle...
  4. Paolini C, Quarta M, Nori A, Boncompagni S, Canato M, Volpe P, et al. Reorganized stores and impaired calcium handling in skeletal muscle of mice lacking calsequestrin-1. J Physiol. 2007;583:767-84 pubmed
    ..Taken together these results demonstrate that CS1 is essential for the normal development of the SR and its calcium release units and for the storage and release of appropriate amounts of SR Ca(2+). ..
  5. Protasi F, Paolini C, Dainese M. Calsequestrin-1: a new candidate gene for malignant hyperthermia and exertional/environmental heat stroke. J Physiol. 2009;587:3095-100 pubmed publisher
    ..In our laboratory we have recently characterized a novel knockout model lacking skeletal muscle calsequestrin (CASQ1), a SR Ca(2+)-binding protein that modulates RyR1 function, and investigated whether these mice present a MH/EHS-..
  6. Zhao X, Min C, Ko J, Parness J, Kim D, Weisleder N, et al. Increased store-operated Ca2+ entry in skeletal muscle with reduced calsequestrin-1 expression. Biophys J. 2010;99:1556-64 pubmed publisher
    ..Previously we showed that calsequestrin-1 (CSQ1) participated in retrograde regulation of SOCE in cultured skeletal myotubes...
  7. 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
    ..Here we describe the identification and functional characterization of a C. elegans calsequestrin gene (csq-1). CSQ-1 shows moderate similarity (50% similarity, 30% identity) to rabbit skeletal calsequestrin...
  8. 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
    ..The study revealed a surprisingly modest loss of Ca(2+) storage capacity in null cells, which may reflect concurrent changes, rather than detract from the physiological importance of calsequestrin...
  9. Paolini C, Quarta M, D Onofrio L, Reggiani C, Protasi F. Differential effect of calsequestrin ablation on structure and function of fast and slow skeletal muscle fibers. J Biomed Biotechnol. 2011;2011:634075 pubmed publisher
    ..The results presented in this paper unmask a differential effect of CASQ1&2 ablation in fast versus slow fibers...

More Information


  1. Nakamura T, Jeromin A, Mikoshiba K, Wakabayashi S. Neuronal calcium sensor-1 promotes immature heart function and hypertrophy by enhancing Ca2+ signals. Circ Res. 2011;109:512-23 pubmed publisher
    ..NCS-1 is an important regulator of immature heart function and hypertrophy, and it functions in part by promoting IP(3)R function, followed by CaMKII-dependent signal activation. ..
  2. Rouger K, Le Cunff M, Steenman M, Potier M, Gibelin N, Dechesne C, et al. Global/temporal gene expression in diaphragm and hindlimb muscles of dystrophin-deficient (mdx) mice. Am J Physiol Cell Physiol. 2002;283:C773-84 pubmed
    ..This finding should be taken under consideration for the interpretation of future experiments using mdx mice as a model for therapeutic assays. ..
  3. Lionikas A, Blizard D, Gerhard G, Vandenbergh D, Stout J, Vogler G, et al. Genetic determinants of weight of fast- and slow-twitch skeletal muscle in 500-day-old mice of the C57BL/6J and DBA/2J lineage. Physiol Genomics. 2005;21:184-92 pubmed
    ..In summary, muscle weight in B6/D2 lineage is affected by a polygenic system that has variable influences at different ages, between males and females, and across muscles in a manner independent of muscle type. ..
  4. Aronow B, Toyokawa T, Canning A, Haghighi K, Delling U, Kranias E, et al. Divergent transcriptional responses to independent genetic causes of cardiac hypertrophy. Physiol Genomics. 2001;6:19-28 pubmed
    ..transgenic mouse hypertrophy models [protein kinase C-epsilon activation peptide (PsiepsilonRACK), calsequestrin (CSQ), calcineurin (CN), and Galpha(q)] were compared by DNA microarray analyses using the approximately 8,800 genes ..
  5. Manno C, Sztretye M, Figueroa L, Allen P, Rios E. Dynamic measurement of the calcium buffering properties of the sarcoplasmic reticulum in mouse skeletal muscle. J Physiol. 2013;591:423-42 pubmed publisher
    ..Free [Ca(2+)](SR) was measured using the cameleon D4cpv-Casq1. In 34 wild-type (WT) cells average B during the depolarization (ON phase) was 157 (SEM 26), implying that of 157 ..
  6. Olojo R, Ziman A, Hernández Ochoa E, Allen P, Schneider M, Ward C. Mice null for calsequestrin 1 exhibit deficits in functional performance and sarcoplasmic reticulum calcium handling. PLoS ONE. 2011;6:e27036 pubmed publisher
    ..Much attention has been focused on calsequestrin (CASQ1) and its role in SR Ca(2+) buffering as well as its potential for modulating RyR1, the L-type Ca(2+) channel (..
  7. Yarotskyy V, Protasi F, Dirksen R. Accelerated activation of SOCE current in myotubes from two mouse models of anesthetic- and heat-induced sudden death. PLoS ONE. 2013;8:e77633 pubmed publisher
    ..anesthetic- and heat-induced sudden death: 1) type 1 ryanodine receptor (RyR1) knock-in mice (Y524S/+) and 2) calsequestrin 1 and 2 double knock-out (dCasq-null) mice...
  8. Seth M, Li T, Graham V, Burch J, Finch E, Stiber J, et al. Dynamic regulation of sarcoplasmic reticulum Ca(2+) stores by stromal interaction molecule 1 and sarcolipin during muscle differentiation. Dev Dyn. 2012;241:639-47 pubmed publisher
    ..These results suggest that SOCE regulates SR/ER specialization and that SLN and STIM1 act in opposing fashions to govern SOCE during myogenesis. ..
  9. Kinnunen S, Mänttäri S. Specific effects of endurance and sprint training on protein expression of calsequestrin and SERCA in mouse skeletal muscle. J Muscle Res Cell Motil. 2012;33:123-30 pubmed publisher
    ..The present study demonstrates the specific effects of different training regimens on CSQ isoform 1 (CSQ1, the primary isoform) and SR Ca²?-ATPase (SERCA1, 2) expression in various skeletal muscles of mouse...
  10. Ferguson D, Dangott L, Schmitt E, Vellers H, Lightfoot J. Differential skeletal muscle proteome of high- and low-active mice. J Appl Physiol (1985). 2014;116:1057-67 pubmed publisher
    ..g., annexin A6, P = 0.0031; calsequestrin 1; P = 0...
  11. Hagiwara N, Ma B, Ly A. Slow and fast fiber isoform gene expression is systematically altered in skeletal muscle of the Sox6 mutant, p100H. Dev Dyn. 2005;234:301-11 pubmed
    ..Together with our earlier report, demonstrating early postnatal muscle defects in the Sox6 null-p100H mutant, the present results suggest that Sox6 likely plays an important role in muscle development...
  12. Ang S, Uebersohn A, Spencer C, Huang Y, Lee J, Ge K, et al. KMT2D regulates specific programs in heart development via histone H3 lysine 4 di-methylation. Development. 2016;143:810-21 pubmed publisher
    ..Our findings indicate that KMT2D is essential for regulating cardiac gene expression during heart development primarily via H3K4 di-methylation. ..
  13. Nori A, Bortoloso E, Frasson F, Valle G, Volpe P. Vesicle budding from endoplasmic reticulum is involved in calsequestrin routing to sarcoplasmic reticulum of skeletal muscles. Biochem J. 2004;379:505-12 pubmed
    ..It also appears that CS routing from ER to SR does not involve classical secretory pathways through ER-Golgi intermediate compartments, cis -medial Golgi and trans -Golgi network. ..
  14. 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. ..
  15. Kibar Z, Vogan K, Groulx N, Justice M, Underhill D, Gros P. Ltap, a mammalian homolog of Drosophila Strabismus/Van Gogh, is altered in the mouse neural tube mutant Loop-tail. Nat Genet. 2001;28:251-5 pubmed
    ..Ltap is expressed broadly in the neuroectoderm throughout early neurogenesis and is altered in two independent Lp alleles, identifying this gene as a strong candidate for Lp. ..
  16. 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...
  17. Mosca B, Eckhardt J, Bergamelli L, Treves S, Bongianino R, De Negri M, et al. Role of the JP45-Calsequestrin Complex on Calcium Entry in Slow Twitch Skeletal Muscles. J Biol Chem. 2016;291:14555-65 pubmed publisher
    We exploited a variety of mouse models to assess the roles of JP45-CASQ1 (CASQ, calsequestrin) and JP45-CASQ2 on calcium entry in slow twitch muscles...
  18. Scorzeto M, Giacomello M, Toniolo L, Canato M, Blaauw B, Paolini C, et al. Mitochondrial Ca2+-handling in fast skeletal muscle fibers from wild type and calsequestrin-null mice. PLoS ONE. 2013;8:e74919 pubmed publisher
    ..The differences in structure and in mitochondrial function observed relative to WT may represent compensatory mechanisms for the disease-related reduction of calcium storage capacity of the SR and/or SR Ca(2+)-leakage. ..
  19. Dilg D, Saleh R, Phelps S, Rose Y, Dupays L, Murphy C, et al. HIRA Is Required for Heart Development and Directly Regulates Tnni2 and Tnnt3. PLoS ONE. 2016;11:e0161096 pubmed publisher
    ..Thus, we show here that HIRA has locus-specific effects on gene expression and that histone chaperone activity is vital for normal heart development, impinging on pathways regulated by an established cardiac transcription factor. ..
  20. Park K, Goo J, Chung H, Kim H, Kim D, Park W. Cloning of the genes encoding mouse cardiac and skeletal calsequestrins: expression pattern during embryogenesis. Gene. 1998;217:25-30 pubmed
    ..The expression of the skeletal calsequestrin in the myotome is regulated probably by myogenin, a myogenic regulatory gene. ..
  21. Mosca B, Delbono O, Laura Messi M, Bergamelli L, Wang Z, Vukcevic M, et al. Enhanced dihydropyridine receptor calcium channel activity restores muscle strength in JP45/CASQ1 double knockout mice. Nat Commun. 2013;4:1541 pubmed publisher
    ..1 and the sarcoplasmic reticulum Ca(2+) storage protein calsequestrin (CASQ1). Here we show that JP45 and CASQ1 strengthen skeletal muscle contraction by modulating Ca(v)1.1 channel activity...
  22. Tomasi M, Canato M, Paolini C, Dainese M, Reggiani C, Volpe P, et al. Calsequestrin (CASQ1) rescues function and structure of calcium release units in skeletal muscles of CASQ1-null mice. Am J Physiol Cell Physiol. 2012;302:C575-86 pubmed publisher
    ..composition of sarcoplasmic reticulum (SR) are altered in fast-twitch skeletal muscles of calsequestrin-1 (CASQ1)-null mice...
  23. Gregory K, Ginsburg K, Bodi I, Hahn H, Marreez Y, Song Q, et al. Histidine-rich Ca binding protein: a regulator of sarcoplasmic reticulum calcium sequestration and cardiac function. J Mol Cell Cardiol. 2006;40:653-65 pubmed
    ..Collectively, these data suggest that HRC may be an integral regulatory protein involved in cardiac muscle SR Ca uptake and Ca homeostasis. ..
  24. Radzyukevich T, Moseley A, Shelly D, Redden G, Behbehani M, Lingrel J, et al. The Na(+)-K(+)-ATPase alpha2-subunit isoform modulates contractility in the perinatal mouse diaphragm. Am J Physiol Cell Physiol. 2004;287:C1300-10 pubmed
    ..They further indicate that the alpha2-isoform contributes significantly less at rest than expected from its proportional content but can modulate contractility during muscle contraction. ..
  25. Bataille N, Schmitt N, Aumercier Maes P, Ollivier B, Lucas Heron B, Lestienne P. Molecular cloning of human calmitine, a mitochondrial calcium binding protein, reveals identity with calsequestrine. Biochem Biophys Res Commun. 1994;203:1477-82 pubmed
    The cDNA of a mitochondrial calcium binding protein, "calmitine", has been cloned from a human skeletal muscle cDNA library. One cDNA of 1.8 kb has been isolated and sequenced...
  26. Ferretti R, Marques M, Pertille A, Santo Neto H. Sarcoplasmic-endoplasmic-reticulum Ca2+-ATPase and calsequestrin are overexpressed in spared intrinsic laryngeal muscles of dystrophin-deficient mdx mice. Muscle Nerve. 2009;39:609-15 pubmed publisher
    ..The results further support the concept that abnormal Ca(2+)-handling is involved in dystrophinopathies. Muscle Nerve, 2009...
  27. Murdoch J, Doudney K, Paternotte C, Copp A, Stanier P. Severe neural tube defects in the loop-tail mouse result from mutation of Lpp1, a novel gene involved in floor plate specification. Hum Mol Genet. 2001;10:2593-601 pubmed
    ..Loss of Lpp1 function disrupts neurulation by permitting more extensive floor plate induction by Shh, thereby inhibiting midline bending of the neural plate during initiation of neurulation. ..
  28. Manno C, Figueroa L, Gillespie D, Fitts R, Kang C, Franzini Armstrong C, et al. Calsequestrin depolymerizes when calcium is depleted in the sarcoplasmic reticulum of working muscle. Proc Natl Acad Sci U S A. 2017;114:E638-E647 pubmed publisher
    ..A channel closing switch operated by calsequestrin depolymerization will limit depletion, thereby preventing full disassembly of the polymeric calsequestrin network and catastrophic structural changes in the SR. ..
  29. Michelucci A, Paolini C, Boncompagni S, Canato M, Reggiani C, Protasi F. Strenuous exercise triggers a life-threatening response in mice susceptible to malignant hyperthermia. FASEB J. 2017;31:3649-3662 pubmed publisher
    ..models that carry a mutation in ryanodine receptor type-1 (RYR1Y522S/WT) and calsequestrin-1 knockout (CASQ1-null), 2 proteins that control Ca2+ release in skeletal muscle...