Results 101 to 110 of about 10,061 (229)
Frataxin Is Localized to Both the Chloroplast and Mitochondrion and Is Involved in Chloroplast Fe-S Protein Function in Arabidopsis. [PDF]
PLoS ONE, 2015 Frataxin plays a key role in eukaryotic cellular iron metabolism, particularly in mitochondrial heme and iron-sulfur (Fe-S) cluster biosynthesis. However, its precise role has yet to be elucidated. In this work, we studied the subcellular localization of
Valeria R Turowski +8 more
doaj +1 more source
Iron–sulfur clusters: from metals through mitochondria biogenesis to disease [PDF]
, 2018 Iron–sulfur clusters are ubiquitous inorganic co-factors that contribute to a wide range of cell pathways including the maintenance of DNA integrity, regulation of gene expression and protein translation, energy production, and antiviral response ...
Cardenas-Rodriguez, Mauricio +2 more
core +1 more source
Plant Biology, EarlyView.Transcript correlation analysis allowed the identification of several key nodes in the complex regulatory network of plant iron metabolism. Abstract Arabidopsis thaliana was the first plant genome to be fully sequenced, almost a quarter of a century ago, thanks to The Arabidopsis Genome Initiative, with contributions from scientists worldwide.
I. Murgia, P. Morandini
wiley +1 more source
Drug Repositioning in Friedreich Ataxia
Frontiers in Neuroscience, 2022 Friedreich ataxia is a rare neurodegenerative disorder caused by insufficient levels of the essential mitochondrial protein frataxin. It is a severely debilitating disease that significantly impacts the quality of life of affected patients and reduces ...
Alessandra Rufini +6 more
doaj +1 more source
Hypoxia Rescues Frataxin Loss by Restoring Iron Sulfur Cluster Biogenesis.
Cell, 2019 Friedreich's ataxia (FRDA) is a devastating, multisystemic disorder caused by recessive mutations in the mitochondrial protein frataxin (FXN). FXN participates in the biosynthesis of Fe-S clusters and is considered to be essential for viability.
Tslil Ast +4 more
semanticscholar +1 more source
Frataxin levels in peripheral tissue in Friedreich ataxia [PDF]
Annals of Clinical and Translational Neurology, 2015 Friedreich ataxia (FRDA) is an autosomal recessive ataxia resulting from mutations in the frataxin gene (FXN). Such mutations, usually expanded guanine-adenine-adenine (GAA) repeats, give rise to decreased levels of frataxin protein in both affected and unaffected tissues.
Lazaropoulos, Michael +19 more
openaire +3 more sources
Iron metabolism in trypanosomatids, and its crucial role in infection. [PDF]
, 2010 Iron is almost ubiquitous in living organisms due to the utility of its redox chemistry. It is also dangerous as it can catalyse the formation of reactive free radicals - a classical double-edged sword.
Kelly, JM, Taylor, MC
core +1 more source
Nerve Ultrasound in Patients With Friedreich Ataxia
Muscle &Nerve, Volume 73, Issue 3, Page 395-402, March 2026.ABSTRACT Introduction/Aims Nerve enlargement has been reported in patients with Friedreich ataxia (FRDA). The underlying cause remains unclear, and both inflammatory processes and dysmyelination have been suggested as potential mechanisms. This study was aimed at assessing nerve morphology with high‐resolution ultrasound, to identify and describe ...
Katharina Kneer +9 more
wiley +1 more source
Biophysical characterisation of the recombinant human frataxin precursor [PDF]
FEBS Open Bio, 2018 Friedreich's ataxia is a disease caused by a decrease in the levels of expression or loss of functionality of the mitochondrial protein frataxin (FXN). The development of an active and stable recombinant variant of FXN is important for protein replacement therapy.
Ignacio Hugo Castro +9 more
openaire +6 more sources
Frontiers in Pharmacology, 2014 Friedreich ataxia (FRDA) is the most common recessive ataxia in the Caucasian population and is characterized by a mixed spinocerebellar and sensory ataxia frequently associating cardiomyopathy.
Alain eMartelli, Helene ePuccio
doaj +1 more source