Disease Models

 

Disease Models

Recapitulate inherited diseases in human engineered cardiac tissues

In vitro disease models enable mutation and patient-specific disease phenotyping and assessment of therapies in the context of human cells. Propria engineers and profiles hEHTs from human induced pluripotent stem cell (hiPSC)-derived cardiomyocytes harboring disease-associated mutations. 

ventricular_vs_R403Q

Engineer the disease model:

  • Start with our lines: In-house lines of the hypertrophic cardiomyopathy mutation MYH7 R403Q and isogenic control
  • Send us hiPSCs: Prorpia differentiates customer hiPSC lines to cardiomyocytes to form hEHTs
  • Send us cardiomyocytes: Propria uses cryopreserved cells to form hEHTs

Understand the phenotype: 

  • Baseline contractile characteristics
  • Length-dependent, frequency-dependent, compound-dependent deficits of disease hEHTs

Assess therapeutic efficacy: 

  • Chronic or acute small molecule treatment
  • Oligonucleotide therapies, including AAVs, ASOs, and siRNAs

 

Click the link below to view data from Propria's MYH7 R403Q hEHTs, presented at the 2023 Heart Development & Disease Keystone Symposium:

Recapitulating Hypertrophic Cardiomyopathy in MyoPod® 3D Human Engineered Heart Tissues 

 

 

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