Article
Insights on the conformational dynamics of human frataxin through modifications of loop-1.
Archives of biochemistry and biophysics - 15 Dec 2017
Noguera Martín E, Aran Martín, Smal Clara, Vazquez Diego S, Herrera María Georgina, Roman Ernesto A, Alaimo Nadine, Gallo Mariana, Santos Javier
Abstract excerpt
Human frataxin (FXN) is a highly conserved mitochondrial protein involved in iron homeostasis and activation of the iron-sulfur cluster assembly. FXN deficiency causes the neurodegenerative disease Friedreich's Ataxia. Here, we investigated the effect of alterations in loop-1, a stretch presumably essential for FXN function, on the conformational stability and dynamics of the native state. We generated four...
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