MyCell Cardiomyocytes DCM LMNA L35P, 01016

Disease Model

Kit Size

Catalog #: R1153
Catalog #: R1146

Cells Only

Catalog #: C1164
Catalog #: C1166

Dilated Cardiomyopathy differentiated from human iPS cells, frozen

From
$795.00

Isogenic Control

Kit Size

Catalog #: R1154
Catalog #: R1147

Cells Only

Catalog #: C1166
Catalog #:

Dilated Cardiomyopathy differentiated from human iPS cells, frozen

From
$795.00
Catalog # GCMCL35P01016

Product Overview

Lamin A/C LMNA mutations are a common cause of dilated cardiomyopathy (DCM). Clinically, DCM is characterized by ventricular dilation concurrent with reduced systolic function and arrhythmias. A missense mutation in the gene encoding the lamin A/C protein (LMNA) results in a change of amino acid 35 from leucine-to-proline (L35P).

To enable researchers to study functional cardiac consequences, somatic cells from an individual carrying the LMNA L35P mutation were reprogrammed to generate MyCell® Cardiomyocytes DCM (L35P), 01016. In addition, an isogenic control, MyCell Cardiomyocytes Corrected DCM (L35P), was generated using genome engineering strategies to correct the mutation. MyCell Cardiomyocytes (L35P) display classic hallmarks of dilated cardiomyopathy including abnormal contractile properties (i.e. reduced contraction amplitude).

Best-in-Class Biology

MyCell Cardiomyocytes (L35P) and its isogenic control (L35P Corrected) exhibit the relevant biology and functionality to advance research and preclinical studies for devastating congenital muscular dystrophies, laminopathies, and cardiomyopathies.

  • 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 MyCell Cardiomyocytes (L35P) and Isogenic Control

Representative phase contrast images (20X) of isogenic control and MyCell Cardiomyocytes (L35P) reveal comparable structure when plated at 20,000 cells per well in 96-well format. (DIV14)

Figure 1: Morphology Assessment

Figure 2: Contractility Measurements (Impedance)

Contractility Measurements (Impedance)

Contractility was monitored by measuring impedance over time. MyCell Cardiomyocytes (L35P) display reduced contraction amplitude relative to isogenic control. (DIV 14)

Calcium Handling Assessment

Representative calcium transients reveal that MyCell Cardiomyocytes (L35P) display increase beat rate relative to isogenic control. (DIV 14)

Figure 3: Calcium Handling Assessment

Publications