Most individuals with Friedreich’s ataxia (FRDA) have both neurologic and cardiac disease which impacts their quality of life. While neurologic symptoms generally are noted earlier in life, cardiac disease is unfortunately the most common cause of death. Both the neurologic and cardiac disease are progressive with generally increasing disability over time in one individual. However, the timing of disease presentation, and progression can vary considerably between different individuals with the disease, which likely result from a combination of genetic and environmental factors. While there has been some progress in understanding the genetic factors that contribute to the differences in neurologic disease severity and progress, our understanding of the genetic factors which influence the development of cardiomyopathy remain limited. This project will focus on trying to identify the genetic factors involved in the early stages of cardiomyopathy by taking advantage of two recent advances, induced pluripotent stem cells (iPSC) which can develop into beating cardiac cells in tissue culture, and gene editing technologies (CRISPR) which allow the systematic evaluation of the importance of genetic factors in the development of cardiomyopathy. If successful, this project will contribute to our understanding of cardiac disease in FRDA and could help identify novel treatment approaches.