Ontology highlight
ABSTRACT:
SUBMITTER: Crombie DE
PROVIDER: S-EPMC5472743 | biostudies-literature | 2017 May
REPOSITORIES: biostudies-literature
Crombie Duncan E DE Curl Claire L CL Raaijmakers Antonia Ja AJ Sivakumaran Priyadharshini P Kulkarni Tejal T Wong Raymond Cb RC Minami Itsunari I Evans-Galea Marguerite V MV Lim Shiang Y SY Delbridge Lea L Corben Louise A LA Dottori Mirella M Nakatsuji Norio N Trounce Ian A IA Hewitt Alex W AW Delatycki Martin B MB Pera Martin F MF Pébay Alice A
Aging 20170501 5
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 derived from three FRDA individuals with characterized GAA repeats. The cells were differe ...[more]