Article

Energy deficit-independent stress response in the Frataxin-depleted heart: evidence that Integrated Stress Response can predominate over mTORC1 activation

2020-06-12

Abstract excerpt

<h4>ABSTRACT</h4> Friedreich’s ataxia (FRDA) is an inherited disorder caused by depletion of frataxin (FXN), a mitochondrial protein required for iron-sulfur cluster (ISC) biogenesis. Cardiac dysfunction is the main cause of death. Yet pathogenesis, and, more generally, how the heart adapts to FXN loss, remain poorly understood, though are expected to be linked to an energy deficit. We modified a transgenic (TG)...

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Literature Corpus work: a0b69f9f-791b-5d9f-bdcb-52bb369ce582
DOI: 10.1101/2020.06.12.148361

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