MyCell Cardiomyocytes HCM MYH7 R403Q, 01178

Disease Model

Kit Size

Catalog #: R1082

Cells Only

Catalog #: C1081
Catalog #: C1119

Hypertrophic Cardiomyopathy differentiated from human iPS cells, frozen

From
$2,700.00

Isogenic Control

Kit Size

Catalog #: R1129

Cells Only

Catalog #: C1119
Catalog #:

Hypertrophic Cardiomyopathy differentiated from human iPS cells, frozen

From
$2,700.00
Catalog # GCMCR403Q01178

Product Overview

Beta (β)-myosin heavy chain (MYH7) R403Q is one of the most common forms and widely studied mutations linked to hypertrophic cardiomyopathy or 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 functional consequences, somatic cells from an individual carrying the HCM MYH7 R403Q mutation were reprogrammed to generate MyCell® Cardiomyocytes (R403Q), 01178. 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

Components:

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Performance Data

Comparison of MyCell Cardiomyocytes (R403Q) and Isogenic Control

MyCell Cardiomyocytes (R403Q) display abnormal electrophysiological properties in multielectrode array (MEA) analysis, including field potential duration (FPD) prolongation, slowed spontaneous beat rate, and increased spike amplitude. (DIV14)

Figure 1: Electrophysiological Analysis

Figure 2: Calcium Handling Assessment

Calcium Handling Assessment

MyCell Cardiomyocytes (R403Q) exhibit diastolic dysfunction consistent with slowed calcium kinetics. Pulse Width Duration = PWD. (DIV 14)

Contractility Measurements (Impedance)

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

Figure 3: Contractility Measurements (Impedance)

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