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BAs act on hepatic FXR and induce SHP expression, thereby inhibiting SREBP-1c and reducing fatty acid synthesis (Watanabe et al., 2004)
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458 Specifically, in response to Frataxin deficiency, the activation of iron-responsive element binding protein 1 (Fe-S protein) increases cellular iron uptake, with the iron being translocated into mitochondria via mitochondrial iron transporters in an attempt to compensate for impaired Fe-S cluster biogenesis
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