iCell Cardiomyocytes DCM LMNA L35P, 01016 and Isogenic Control

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 iCell^® Cardiomyocytes DCM (L35P), 01016. In addition, an isogenic control, iCell Cardiomyocytes Corrected DCM (L35P), was generated using genome engineering strategies to correct the mutation. iCell Cardiomyocytes (L35P) display classic hallmarks of dilated cardiomyopathy including abnormal contractile properties (i.e. reduced contraction amplitude).

Best-in-Class Biology

iCell 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