CASQ2 Antibody

Code CSB-PA004557LA01HU
Size US$166
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  • Western Blot
    Positive WB detected in: MCF-7 whole cell lysate
    All lanes: CASQ2 antibody at 3µg/ml
    Secondary
    Goat polyclonal to rabbit IgG at 1/50000 dilution
    Predicted band size: 47, 39 kDa
    Observed band size: 47 kDa

  • Immunohistochemistry of paraffin-embedded human skeletal muscle tissue using CSB-PA004557LA01HU at dilution of 1:100

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) CASQ2 Polyclonal antibody
Uniprot No.
Target Names
CASQ2
Alternative Names
AA033488 antibody; AW146219 antibody; Calsequestrin 2 (cardiac muscle) antibody; Calsequestrin 2 fast twitch cardiac muscle antibody; Calsequestrin antibody; Calsequestrin cardiac muscle isoform antibody; Calsequestrin fast twitch cardiac muscle antibody; Calsequestrin-2 antibody; Calsequestrin2 antibody; cardCSQ antibody; Cardiac calsequestrin 2 antibody; cardiac muscle isoform antibody; CASQ 2 antibody; CASQ2 antibody; CASQ2_HUMAN antibody; cCSQ antibody; ESTM52 antibody; FLJ26321 antibody; FLJ93514 antibody; PDIB2 antibody
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Calsequestrin-2 protein (219-300AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated

The CASQ2 Antibody (Product code: CSB-PA004557LA01HU) is Non-conjugated. For CASQ2 Antibody with conjugates, please check the following table.

Available Conjugates
Conjugate Product Code Product Name Application
HRP CSB-PA004557LB01HU CASQ2 Antibody, HRP conjugated ELISA
FITC CSB-PA004557LC01HU CASQ2 Antibody, FITC conjugated
Biotin CSB-PA004557LD01HU CASQ2 Antibody, Biotin conjugated ELISA
Clonality
Polyclonal
Isotype
IgG
Purification Method
>95%, Protein G purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Tested Applications
ELISA, WB, IHC
Recommended Dilution
Application Recommended Dilution
WB 1:500-1:5000
IHC 1:20-1:200
Troubleshooting and FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

Customer Reviews and Q&A

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Target Background

Function
Calsequestrin is a high-capacity, moderate affinity, calcium-binding protein and thus acts as an internal calcium store in muscle. Calcium ions are bound by clusters of acidic residues at the protein surface, especially at the interface between subunits. Can bind around 60 Ca(2+) ions. Regulates the release of lumenal Ca(2+) via the calcium release channel RYR2; this plays an important role in triggering muscle contraction. Plays a role in excitation-contraction coupling in the heart and in regulating the rate of heart beats.
Gene References into Functions
  1. We show for the first time a heterozygous CASQ2 variant causing autosomal dominant CPVT in a large family with a severe phenotype. PMID: 27157848
  2. a direct interaction exists between RyR2 and CSQ2, is reported. PMID: 27609834
  3. induced Pluripotent Stem Cell-derived cardiomyocytes are useful for investigating the similarities/differences in the pathophysiological consequences of RyR2 versus CASQ2 mutations underlying Catecholaminergic polymorphic ventricular tachycardia. PMID: 26153920
  4. Mutations in the MYBPC3 and CASQ2 genes and six combinations between loci in the MYBPC3, MYH7 and CASQ2 genes were responsible for cardiomyopathy risk in a studied cohort. PMID: 25892673
  5. We observed association between a CASQ2 polymorphism and SCA due to VA in patients with CAD adjusting for CHF and independent associations between CASQ2 SNPs and CHF adjusting for SCA. PMID: 24444446
  6. Molecular analysis of the CASQ2 gene in 43 probands with Catecholaminergic polymorphic ventricular tachycardia were performed and eight mutations in five patients, were identified. PMID: 21618644
  7. Genetic background of catecholaminergic polymorphic ventricular tachycardia in Japan. PMID: 23595086
  8. In a consanguineous family, a novel homozygous CASQ2 mutation (p.L77P) was identified in a child with CPVT who required implantation of a cardioverter defibrillator due to episodes of syncope while on medical therapy PMID: 22650415
  9. A review of the physiology of Casq2 in cardiac Ca2+ handling and discuss pathophysiological mechanisms that lead to catecholaminergic polymorphic ventricular tachycardia caused by CASQ2 mutations. PMID: 22421959
  10. patients with CASQ2-associated CPVT should be recommended to receive ICDs to prevent sudden death when medical therapy is not effective. PMID: 22481011
  11. Aspartate to histidine casq2 mutation causes arrhythmia in cardiomyocytes generated from catecholaminergic polymorphic ventricular tachycardia patients. PMID: 22050625
  12. Ca(2+) and JNT-dependent disassembly of the CSQ2 polymer PMID: 22123818
  13. Two causative genes of CPVT have been identified: RYR2, encoding the cardiac ryanodine receptor (RyR2) Ca(2+) release channel, and CASQ2, encoding cardiac calsequestrin. Their mutation have been found in 60% of patients with CPVT. PMID: 21872879
  14. Common variations in or near CASQ2, GPD1L, and NOS1AP are associated with increased risk of sudden cardiac death in patients with coronary artery disease PMID: 21685173
  15. Studies identified two phosphorylation sites, Ser(385) and Ser(393 in hCASQ2 by mass-spectroscopy. PMID: 21416293
  16. Catecholaminergic polymorphic ventricular tachycardia (CPTV) mutations modify CASQ2 behaviour, including folding, aggregation/polymerization and selectivity towards Ca2+. PMID: 21265816
  17. up-regulation of casq2 gene in the thyroid of patients with Graves' Hyperthyroidism may lead to the production of autoantibodies and sensitized T-lymphocytes, which cross-react with calsequestrin of patients who develop ophthalmopathy. PMID: 20039900
  18. A regulatory role of CASQ2 on cytosolic Ca(2+) and hERG channels which may contribute to the etiology of CPVT. PMID: 21063088
  19. The human CASQ2 mutation K206N is associated with hyperglycosylation and altered cellular calcium handling. PMID: 20302875
  20. missense mutation in a highly conserved region of CASQ2 is associated with autosomal recessive catecholamine-induced polymorphic ventricular tachycardia in Bedouin families from Israel PMID: 11704930
  21. calsequestrin 2 mutations causes severe forms of catecholaminergic polymorphic ventricular tachycardia PMID: 12386154
  22. missense mutation in the CASQ2 gene is associated with autosomal-recessive CPVT(catecholamine-induced polymorphic ventricular tachycardia). PMID: 12732448
  23. A missense mutation in a highly conserved region of CASQ2 is associated with autosomal recessive catecholamine-induced polymorphic ventricular tachycardia in Bedouin families from Israel. PMID: 12858557
  24. Intracellular Ca2+ cycling in normal heart relies on intricate interplay of CASQ2 with proteins of RyR2 channel complex, and disruption of these interactions can lead to cardiac arrhythmia. PMID: 16601229
  25. CASQ2 mutations identified in polymorphic ventricular tachycardia create distinct abnormalities that lead to abnormal intracellular calcium regulation, thus facilitating the development of tachyarrhythmias. PMID: 16908766
  26. CSQ was a highly phosphorylated protein with a glycan structure predictive of ER-retained proteins. PMID: 17045261
  27. A variant was identified in CASQ2. PMID: 17655857
  28. Data show that all three CPVT-related missense mutations lead to significant reduction in Ca2+-binding capacity and scattering experiments confirm that the linear polymerization behavior of CSQ is linked directly to its high-capacity Ca2+ binding. PMID: 17881003
  29. analysis of how human and rat CASQ2 ventricular tachycardia-related mutations R33Q and L167H alter calcium sensitivity PMID: 18399795
  30. study concludes CASQ2 in the sarcoplasmic reticulum (SR )determines magnitude & duration of Ca release from each SR terminal; 2 CPVT-inducing CASQ2 mutations lead to increased diastolic SR Ca release events and exhibit a similar CPVT disease phenotype PMID: 18469084
  31. A novel mutation of F189L in the CASQ2 gene was identified in families with catecholaminergic polymorphic ventricular tachycardia. PMID: 18543230
  32. REVIEW of Casq2 mutations that cause catecholaminergic polymorphic ventricular tachycardia and their effects on Casq2 function and Ca handling PMID: 18669926
  33. Facilitated maturation of Ca2+ handling properties of human embryonic stem cell-derived cardiomyocytes by calsequestrin expression. PMID: 19357236
  34. Data suggest that calsequestrin (CSQ)2 facilitates Ca(2+) release through RyR2 during systole, while CSQ1 curtails RyR1 opening to maintain Ca(2+) and allow repeated release/ graded activation with increased stimulation frequency. PMID: 19376574
  35. Cardiac and fatal or near-fatal events were not rare in both catecholaminergic polymorphic ventricular tachycardia RYR2 and a CASQ2 mutation probands and affected family members during the long-term follow-up PMID: 19398665

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Involvement in disease
Ventricular tachycardia, catecholaminergic polymorphic, 2 (CPVT2)
Subcellular Location
Sarcoplasmic reticulum lumen.
Protein Families
Calsequestrin family
Database Links

HGNC: 1513

OMIM: 114251

KEGG: hsa:845

STRING: 9606.ENSP00000261448

UniGene: Hs.57975

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