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iCell Cardiomyocytes CPVT (E2311D)

Model Catecholaminergic Polymorphic Ventricular Tachycardia in vitro

Cardiac Ryanodine Receptor (RYR2) is one of the most important proteins in cardiomyocyte physiology and calcium-induced calcium release. Mutations in RYR2 are a common cause of Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT). Clinically, CPVT is characterized by life-threatening arrhythmias which may present during exercise or stress.

A missense mutation in the gene encoding Cardiac Ryanodine Receptor (RYR2) gives rise to CPVT Type 1 resulting in a change of amino acid 2311 from Glutamic Acid-to-Aspartic Acid (E2311D). The pathobiology of this mutation remains generally poorly understood.

To enable investigation of the functional consequences of the mutation, the E2311D mutation was engineered in an otherwise apparently healthy, normal donor background, 01434. The use of genome engineering strategies to generate this mutation results in an isogenic pair that is vital for analysis.

iCell® Cardiomyocytes (E2311D) display abnormal cardiomyocyte function consistent with CPVT mutations associated with the cardiac ryanodine receptor.

Best-in-Class Biology

iCell Cardiomyocytes (E2311D), 01434, and its isogenic control (iCell Cardiomyocytes, 01434) exhibit the relevant biology and functionality to advance research and preclinical studies for life-threatening arrhythmias:

  • Fully differentiated, >90% pure cardiomyocytes
  • Expression of relevant cardiac markers (e.g. cTNT, ACTN2)
  • Isogenic control available

Comparison of iCell Cardiomyocytes (E2311D) and Isogenic Control

Figure 1: Electrophysiological Analysis
iCell Cardiomyocytes (E2311D) display abnormal electrophysiological properties in multielectrode array (MEA) analysis, including field potential (FDP) prolongation, increased beat period, and increased conduction velocity relative to isogenic control. *P<0.05 (DIV14)

Figure 2: Calcium Handling Assessment
Representative calcium transients reveal that iCell Cardiomyocytes (E2311D) display comparable amplitude and reduced beat rate relative to isogenic control. *P<0.05 (DIV 14)

Figure 3: Contractility Measurements (Impedance)
Contractility was monitored by measuring impedance over time. iCell Cardiomyocytes (E2311D) display comparable contraction amplitude, reduced beat rate, and increased beat rate irregularity relative to isogenic control. *P<0.05 (DIV 14)

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    Catalog # Description Quantity Price
    R1155 iCell Cardiomyocytes CPVT (E2311D) Kit, 01434

    Each kit contains:

    • Cryopreserved iCell Cardiomyocytes CPVT (E2311D), 01434
    • 30 ml Plating Medium
    • 2 x 100 ml Maintenance Medium
    • User’s Guide
    ≥4 x 106
    Viable Cells/Vial
    $2250
    Inquire About Pricing & Availability
    R1007 iCell Cardiomyocytes Kit, 01434

    Each kit contains:

    • Cryopreserved iCell Cardiomyocytes, 01434
    • 30 ml Plating Medium
    • 2 x 100 ml Maintenance Medium
    • User’s Guide
    ≥4 x 106
    Viable Cells/Vial
    $1545
    Inquire About Pricing & Availability
    M1003 iCell Cardiomyocytes Maintenance Medium

    Serum-based medium with pH indicator

    100 ml (1X) $125
    Inquire About Pricing & Availability
    M1004 iCell Cardiomyocytes Maintenance Medium

    Serum-based medium with pH indicator

    500 ml (5X) $350
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    R1151 iCell Cardiomyocytes Media Kit

    Each kit contains:

    • M1001 30ml iCell Cardiomyocytes Plating Medium
    • M1003 100ml iCell Cardiomyocytes Maintenance Medium
    1 Kit $375
    Inquire About Pricing & Availability