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MyCell® Cardiomyocytes (R403Q)

Study the Most Prevalent Hypertrophic Cardiomyopathy Mutation in Human Cardiomyocytes

Beta (β)-myosin heavy chain (MYH7) R403Q is one of the most common forms and widely studied mutations linked to hypertrophic cardiomyopathy (HCM). Clinically, HCM is characterized by ventricular wall thickening as a result of enlarged cardiomyocytes, preserved ejection fraction concurrent with diastolic dysfunction, and arrhythmias. A missense mutation in the gene encoding the beta myosin heavy chain protein (MYH7) results in a change of amino acid 403 from arginine-to-glutamine (R403Q). The pathobiology of this mutation remains generally poorly understood.
To enable researchers to study the functional consequences of the R403Q mutation, somatic cells from an individual carrying the HCM MYH7 R403Q mutation were reprogrammed to generate MyCell® Cardiomyocytes (R403Q), 00178. In addition, an isogenic control, MyCell Cardiomyocytes MYH7 (R403Q) Corrected, was generated using genome engineering strategies to correct the mutation. MyCell Cardiomyocytes (R403Q) display classic hallmarks of hypertrophic cardiomyopathy including abnormal electrophysiological properties, calcium handling and contractile properties.

Best-in-Class Biology

MyCell Cardiomyocytes (R403Q) and its isogenic control (R403Q Corrected) exhibit the relevant biology and functionality to advance research and preclinical studies for devastating cardiomyopathies.

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

Comparison of MyCell Cardiomyocytes (R403Q) and Isogenic Control

Figure 1: Electrophysiological Analysis
MyCell® Cardiomyocytes (R403Q) display abnormal electrophysiological properties in multielectrode array (MEA) analysis, including field potential (FDP) prolongation, slowed spontaneous beat rate and increased spike amplitude. (DIV14)

Figure 2: Calcium Handling Assessment
MyCell® Cardiomyocytes (R403Q) exhibit diastolic dysfunction consistent with slowed calcium kinetics. (DIV 14). Pulse Width Duration = PWD

Figure 3: Contractility Measurements (Impedance)
Contractility was monitored by measuring impedance over time. MyCell® Cardiomyocytes (R403Q) display enhanced contraction and increased arrhythmic events compared to the corrected isogenic control. (DIV 14)

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    Catalog # Description Quantity Price
    R1082 MyCell Cardiomyocytes HCM (R403Q) Kit, 01178

    Each kit contains:

    • Cryopreserved MyCell R403Q Cardiomyocytes
    • 30 ml Plating Medium
    • 2 x 100 ml Maintenance Medium
    • User’s Guide
    ≥4 x 106
    Viable Cells/Vial
    $2250
    Inquire About Pricing & Availability
    R1129 MyCell Cardiomyocytes Corrected HCM (R403Q) Kit, 01178

    Each kit contains:

    • Cryopreserved MyCell Cardiomyocytes Corrected HCM (R403Q isogenic control)
    • 30 ml Plating Medium
    • 2 x 100 ml Maintenance Medium
    • User’s Guide
    ≥4 x 106
    Viable Cells/Vial
    $2250
    Inquire About Pricing & Availability
    R1130 MyCell Cardiomyocytes HCM (R403Q) 01178, Pair

    Each kit contains:

    • Cryopreserved MyCell R403Q Cardiomyocytes
    • Cryopreserved MyCell Cardiomyocytes Corrected HCM (R403Q isogenic control)
    • 2 x 30 ml Plating Medium
    • 4 x 100 ml Maintenance Medium
    • User’s Guide
    2 Vials
    ≥4 x 106
    Viable Cells/Vial
    $4000
    Inquire About Pricing & Availability