Adaptation of the heart to frataxin depletion: evidence that integrated stress response can predominate over mTORC1 activation

Abstract Friedreich’s ataxia 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, remains poo...

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Veröffentlicht in:	Human molecular genetics 2021-09, Vol.33 (8), p.637-654
Hauptverfasser:	Vásquez-Trincado, César, Patel, Monika, Sivaramakrishnan, Aishwarya, Bekeová, Carmen, Anderson-Pullinger, Lauren, Wang, Nadan, Tang, Hsin-Yao, Seifert, Erin L
Format:	Artikel
Sprache:	eng
Online-Zugang:	Volltext
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