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iCell Cardiomyocytes CPVT RYR2 E2311D, 01434
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
Components:
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Need Help With This Product?
Our specialists are here to help you find the best product for your application.
Our regular business hours are 9:00am to 5:00pm Central Time (USA)
Technical Docs
PROTOCOLS
Application Notes
Applying Transfection Technologies to Create Novel Screening Models - iCell Cardiac Products
Application Notes
Assaying Caspase Activity & Apoptosis
Application Notes
Assaying Cell Viability
Application Notes
Assaying Cytotoxicity
Application Notes
Assaying Mitochondrial Membrane Potential
Application Protocols
Culturing and Assaying Calcium Transients of 3D Cardiac Tri-Culture Microtissues
Application Protocols
Dissociating Cardiomyocytes by Enzymatic Treatment
Application Protocols
Extracting Total RNA
Application Protocols
Immunofluorescent Labeling of iCell Cardiomyocytes
Application Protocols
Measuring Cardiac Activity: Impedance Detection with xCELLigence RTCA Cardio System
Application Protocols
Measuring Cardiac Activity: Intracellular Calcium Flux Detection on the FLIPR Tetra System
Application Protocols
Measuring Cardiac Electrical Activity: Field Potential Detection with Axion Maestro Multielectrode Array
Application Protocols
Measuring Cardiac Electrical Activity: Field Potential Detection with Multielectrode Array
Application Protocols
Measuring Cardiac Electrical Activity: Manual Perforated Patch Clamp
Application Protocols
Modeling Cardiac Hypertrophy: Endothelin-1 Induction with ELISA Analysis
Application Protocols
Modeling Cardiac Hypertrophy: Endothelin-1 Induction with Flow Cytometry Analysis
Application Protocols
Modeling Cardiac Hypertrophy: Endothelin-1 Induction with High Content Analysis
Application Protocols
Modeling Cardiac Hypertrophy: Endothelin-1 Induction with qRT-PCR Analysis
Application Protocols
Modeling Cardiac Ischemia: Hypoxia Induction for Cardioprotection Screening
Application Protocols
Performing Bioenergetic Analysis: Seahorse Bioscience XF96 Extracellular Flux Analyzer
Application Protocols
Using Liposome-mediated Transfection for Gene Delivery
Application Protocols
Using Transfection for siRNA Delivery
USER DOCS
Biosafety Document
Biosafety Document: iCell Cardiomyocytes and iCell Cardiomyocytes2 01434
Biosafety Document
iCell Cardiomyocytes CPVT (E2311D) Biosafety Document
Datasheet
iCell Cardiomyocytes
Safety Data Sheet
iCell Cardiomyocytes Maintenance Medium, iCell Cardiomyocytes Plating Medium Safety Data Sheet - Asia
Safety Data Sheet
iCell Cardiomyocytes Maintenance Medium, iCell Cardiomyocytes Plating Medium Safety Data Sheet - Europe
Safety Data Sheet
iCell Cardiomyocytes Maintenance Medium, iCell Cardiomyocytes Plating Medium Safety Data Sheet - Germany
Safety Data Sheet
iCell Cardiomyocytes Maintenance Medium, iCell Cardiomyocytes Plating Medium Safety Data Sheet - North America
Safety Data Sheet
iCell Cell-Based Products Safety Data Sheet - Asia
Safety Data Sheet
iCell Cell-Based Products Safety Data Sheet - Germany
Safety Data Sheet
iCell Cell-Based Products Safety Data Sheet - North America
User's Guides
iCell Cardiomyocytes
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
Publications
Electronic Cigarette Extract Induced Toxic Effect in iPS-derived Cardiomyocytes Hesham Basma, Swetha Tatineni, Kajari Dhar, Fang Qiu, Stephen Rennard, Brian D. Lowes (2020) BMC Cardiovasc Disord. 20: 357. (2020)
Inhibition of RNA Helicase Activity Prevents Coxsackievirus B3-Induced Myocarditis in Human iPS Cardiomyocytes Yun SH, Shin HH, Ju ES, Lee YJ, Lim BK & Jeon ES (2020) Int. J. Mol. Sci. 21: 3041. (2020)
Selective Induction of Human Autonomic Neurons Enables Precise Control of Cardiomyocyte Beating Takayama Y, Kushige H, Akagi Y, Suzuki Y, Kumagai Y & Kida YS (2020) Scientific Reports 10: 9464 (2020)
Detection of Drug-Induced Torsades de Pointes Arrhythmia Mechanisms Using hiPSC-CM Syncytial Monolayers in a High-Throughput Screening Voltage Sensitive Dye Assay da Rocha AM, Creech J, Thonn E, Mironov S, Herron TJ (2020) Toxicol Sci. 173(2):402-415 (2020)
Multiplexed Optical Sensors in Arrayed Islands of Cells for Multimodal Recordings of Cellular Physiology Christopher A. Werley, Stefano Boccardo, Alessandra Rigamonti, Emil M. Hansson & Adam E. Cohen (2020) Nature Comm. 11: 3881. (2020)
Assessing SSRIs' Effects on Fetal Cardiomyocytes Utilizing Placenta-Fetus Model Arumugasaamy N, Hurley-Novatny A, Lembong J, Kim PCW, Fisher JP. (2019) Acta Biomater. 99:258-268. (2019)
TGF-β Induces a Heart Failure Phenotype via Fibroblasts Exosome Signaling Basma H, Johanson AN, Dhar K, Anderson D, Qiu F, Rennard S, Lowes BD. (2019) Heliyon, 5:10, e02633. (2019)
Resolving the Reversed Rate Effect of Calcium Channel Blockers on Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes and the Impact on in vitro Cardiac Safety Evaluation. Zeng H, Wang J, Clouse H, Lagrutta A, and Sannajust F. Toxicol Sci. (2019) 167(2):573-580. (2019)
Inhibitory Effects of Class 1 Antiarrhythmic Agents on Na+ and Ca2+ Currents of Human iPS Cell-derived Cardiomyocytes Yonemizu S, Masuda K, Kurata Y, Notsu T, Higashi Y, Fukumura K, Li P, Ninomiya H, Miake J, Tsuneto M, Shirayoshi Y, Hisatomea I (2019) Regen Ther 10: 104–111. (2019)
Human Cardiac Organoids for the Modeling of Myocardial Infarction and Drug Cardiotoxicity Richards DJ, Li Y, Kerr CM, Yao J, Beeson GC, Coyle RC, Chen X, Jia J, Damon B, Wilson R, Starr Hazard E, Hardiman G, Menick DR, Beeson CC, Yao H, Ye T, Mei Y (2020) Nat Biomed Eng. 4(4):446-462. (2020)