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Friedreich's ataxia: the vicious circle hypothesis revisited

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Academic year: 2021

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Figure 1 Frataxin function in the mitochondria. The schema illustrates the iron-sulphur cluster (ISC) biosynthesis machinery present in the mitochondrial matrix encompassing the ISCU-NFS1 protein complex associating glutaredoxin 5 (GLRX5) with the frataxin
Figure 2 The vicious circle hypothesis revisited in Friedreich ’ s ataxia. (A) According to the vicious circle hypothesis, frataxin depletion results in impaired iron-sulphur cluster synthesis and/or stability with intramitochondrial accumulation of reacti
Table 1 Consequences of frataxin depletion in various organisms a
Table 2 Cardinal clinical symptoms associated with deficiencies in frataxin, vitamin E, ABCB7 (mitochondrial iron overload), GLRX5 (impaired ISC synthesis) and ISCU (impaired ISC synthesis) a

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