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Copper redox chemistry of plant frataxins
(Elsevier Science Inc, 2018-03)
The presence of a conserved cysteine residue in the C-terminal amino acid sequences of plant frataxins differentiates these frataxins from those of other kingdoms and may be key in frataxin assembly and function. We report ...
Plant frataxin in metal metabolism
(Frontiers Research Foundation, 2018-02)
Frataxin is a highly conserved protein from prokaryotes to eukaryotes. Several functions related to iron metabolism have been postulated for this protein, including Fe-S cluster and heme synthesis, response to oxidative ...
Frataxin Structure and Function
(Springer, 2019)
Mammalian frataxin is a small mitochondrial protein involved in iron sulfur cluster assembly. Frataxin deficiency causes the neurodegenerative disease Friedreich´s Ataxia. Valuable knowledge has been gained on the structural ...
Identification of two frataxin isoforms in Zea mays: Structural and functional studies
(Elsevier Masson, 2017-09)
Frataxin is a ubiquitous protein that plays a role in Fe-S cluster biosynthesis and iron and heme metabolism, although its molecular functions are not entirely clear. In non-photosynthetic eukaryotes, frataxin is encoded ...
Nitric oxide accumulation is required to protect against iron-mediated oxidative stress in frataxin-deficient Arabidopsis plants
(Elsevier Science, 2009-01)
Frataxin is a mitochondrial protein that is conserved throughout evolution. In yeast and mammals, frataxin is essential for cellular iron (Fe) homeostasis and survival during oxidative stress. In plants, frataxin deficiency ...
Human Frataxin Folds Via an Intermediate State. Role of the C-Terminal Region
(Nature Publishing Group, 2016-02)
The aim of this study is to investigate the folding reaction of human frataxin, whose deficiency causes the neurodegenerative disease Friedreich’s Ataxia (FRDA). The characterization of different conformational states would ...
Ferrochelatase activity of plant frataxin
(Elsevier France-Editions Scientifiques Medicales Elsevier, 2019-01)
Frataxin plays a key role in cellular iron homeostasis of different organisms. It is engaged in several activities at the Fe–S cluster assembly machinery and it is also involved in heme biosynthesis. In plants, two genes ...
Nitric Oxide and Frataxin: Two Players Contributing to Keep Cellular Iron Homeostasis.
(Oxford University Press, 2009-06-25)
BackgroundNitric oxide (NO) is a signalling and physiologically active molecule in animals, plants and bacteria. The specificity of the molecular mechanism(s) involved in transducing the NO signal within and between cells ...
Yeast frataxin mutants display decreased superoxide dismutase activity crucial to promote protein oxidative damage
(Elsevier Science Inc, 2010-02-01)
Iron overload is involved in several pathological conditions, including Friedreich ataxia, a disease caused by decreased expression of the mitochondrial protein frataxin. In a previous study, we identified 14 proteins ...
Folding and Dynamics Are Strongly pH-Dependent in a Psychrophile Frataxin
(American Chemical Society, 2019-09)
Protein dynamics, folding, and thermodynamics represent a central aspect of biophysical chemistry. pH, temperature, and denaturant perturbations inform our understanding of diverse contributors to stability and rates. In ...