iCell Cardiomyocytes CPVT RYR2 E2311D, 01434

Disease Model

Kit Size

Catalog #: R1155

Cells Only

Catalog #: C1207
Catalog #: C1207

Catecholaminergic Polymorphic Ventricular Tachycardia differentiated from human iPS cells, frozen

From
$2,700.00

Isogenic Control

Kit Size

Catalog #: R1057
Catalog #: R1132
Catalog #: R1007

Cells Only

Catalog #: C1056
Catalog #: C1006
Catalog #:

Catecholaminergic Polymorphic Ventricular Tachycardia differentiated from human iPS cells, frozen

From
$595.00
Catalog # GCMCE2311D01434

Product Overview

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

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Performance Data

Comparison of iCell Cardiomyocytes (E2311D) and Isogenic Control

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 1: Figure 1: Electrophysiological Analysis

Figure 2: Pharmacological Response to L-type Calcium Channel Agonist

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).

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).

Figure 3: Pharmacological Response to L-type Calcium Channel Antagonist

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