Article
Friedreich's ataxia induced pluripotent stem cell-derived cardiomyocytes display electrophysiological abnormalities and calcium handling deficiency.
Aging - 30 May 2017
Crombie Duncan E, Curl Claire L, Raaijmakers Antonia Ja, Sivakumaran Priyadharshini, Kulkarni Tejal, Wong Raymond Cb, Minami Itsunari, Evans-Galea Marguerite V, Lim Shiang Y, Delbridge Lea, Corben Louise A, Dottori Mirella, Nakatsuji Norio, Trounce Ian A, Hewitt Alex W, Delatycki Martin B, Pera Martin F, Pébay Alice
Abstract excerpt
We sought to identify the impacts of Friedreich's ataxia (FRDA) on cardiomyocytes. FRDA is an autosomal recessive degenerative condition with neuronal and non-neuronal manifestations, the latter including progressive cardiomyopathy of the left ventricle, the leading cause of death in FRDA. Little is known about the cellular pathogenesis of FRDA in cardiomyocytes. Induced pluripotent stem cells (iPSCs) were...
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