Activation of Ferroptosis Pathways in FSHD (IMAGE)
Caption
This study reveals abnormal iron accumulation in the muscles of patients with Facioscapulohumeral Muscular Dystrophy (FSHD) and in mice expressing muscle-specific DUX4 (DUX4-Tg mice). Contrary to expectations, treatments involving iron chelation, an iron-deficient diet, or genetic modifications to inhibit iron uptake did not alleviate but instead worsened the pathology of FSHD in DUX4-Tg mice. Interestingly, supplementation with iron—either through a high-iron diet or intravenous iron administration—led to significant improvements in grip strength and running performance in these mice by suppressing DUX4-activated ferroptosis pathway.
Image by Nakamura et al., originally published in Journal of Clinical Investigation, licensed under CC BY 4.0.
Source: https://www.jci.org/articles/view/181881
Credit
Nakamura et al.
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CC BY