Endoplasmic Reticulum-mitochondria associated membranes (MAMs) are inter-organelle structures involved in the regulation of essential cellular processes, including lipid metabolism and calcium signaling. The present study analyzes calcium management and integrity of MAMs in both unicellular and multicellular models of Friedreich’s ataxia (FRDA). The authors observed that the function of MAMs is compromised in a cellular model of FRDA, which was improved upon treatment with antioxidants. In agreement, promoting mitochondrial calcium uptake was sufficient to restore several defects caused by frataxin deficiency in Drosophila Melanogaster. Remarkably, these findings describe for the first time frataxin as a member of the protein network of MAMs, where it interacts with two of the main proteins implicated in endoplasmic reticulum-mitochondria communication. These results suggest a new role of frataxin, indicate that FRDA goes beyond mitochondrial defects and highlight MAMs as novel therapeutic candidates to improve patients' conditions.

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