The role of frataxin in fission yeast iron metabolism: Implications for Friedreich’s ataxia. Yu Wang, Yiwei Wang, S. Marcus, L.S. Busenlehner; Biochimica et Biophysica Acta (BBA) - General Subjects, Available online 3 July 2014, ISSN 0304-4165, http://dx.doi.org/10.1016/j.bbagen.2014.06.017
This paper provide evidence that suggests dysregulated Fe-S cluster biogenesis is a primary effect of both frataxin overexpression and deficiency as in Friedreich’s ataxia, it also suggests that therapies to increase transcription and translation of endogenous frataxin or to replace exogenous frataxin via gene therapy, must tightly control protein expression since high levels of frataxin are detrimental to the cell in a manner similar to its deficiency.
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