Mitochondrial Calcium Homeostasis in the Pathology and Therapeutic Application in Friedreich's Ataxia

A few drugs targeting Ca2+ homeostasis have shown good preclinical efficacy in the treatment of FRDA. However, the clinical Ca2+ data are very limited. The enhancement of mitochondrial Ca2+ flux, including influx and efflux, benefits FXN-deficient cells in vitro. Further, Ca2+ chelation or the use of CsA to block the opening of the mPTP improves mitochondrial function. The authors demonstrated that the peptide SS-31, by targeting mitochondrial cardiolipin, upregulates FXN expression and improves mitochondrial function and propose that this effect depends on mitochondrial membrane potential and, directly or indirectly, leads to recovery of the Ca2+ flow.

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