Article
Conformational stability, dynamics and function of human frataxin: Tryptophan side chain interplay.
Archives of biochemistry and biophysics - 15 Jan 2022
Espeche Lucía D, Sewell Karl Ellioth, Castro Ignacio H, Capece Luciana, Pignataro María Florencia, Dain Liliana, Santos Javier
Abstract excerpt
In humans, the loss of frataxin results in Friedreich's Ataxia, a neurodegenerative disease, in which a deficit in the iron-sulfur cluster assembly is observed. In this work, we analyzed three frataxin variants in which one tryptophan was replaced by a glycine: W155G, W168G and W173G. As expected, given its localization in the assembly site, W155G was not able to activate the desulfurase activity of the...
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