Tommaso Vannocci, Simone Dinarelli, Marco Girasole, Annalisa Pastore & Giovanni Longo; Sci Rep 9, 19282 (2019) doi:10.1038/s41598-019-55799-z
Accordingly, the disease starts appearing when the frataxin levels are below 30% those of healthy controls and are 10–35% the normal levels in FRDA patients. On the other hand, it was found that frataxin overexpression is not a solution as a therapeutic strategy: overexpression seems to be as toxic as its partial depletion. This observation is perfectly coherent with the current hypothesis that frataxin functions as a regulator of the process of conversion of cysteine into alanine through interaction with the desulfurase central to the machine of the iron sulfur cluster biogenesis machine.
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