Results 211 to 220 of about 10,061 (229)

Oxidative stress-dependent frataxin inhibition mediated alcoholic hepatocytotoxicity through ferroptosis.

Toxicology, 2020
Alcoholic liver disease (ALD) is one of the severe liver diseases, resulting in high morbidity and mortality. However, frataxin, a mitochondrial protein mainly participating in iron homeostasis and oxidative stress, remains uncertain in the pathogenesis ...
Jing-jing Liu, Hui He, Jing Wang, Xiaoping Guo, Hongkun Lin, Huimin Chen, Chunjie Jiang, Li Chen, P. Yao, Yuhan Tang   +9 more
semanticscholar   +1 more source

SS-31 efficacy in a mouse model of Friedreich ataxia by upregulation of frataxin expression.

Human Molecular Genetics, 2021
Friedreich ataxia (FRDA) is a serious hereditary neurodegenerative disease, mostly accompanied with hypertrophic cardiomyopathy, caused by the reduced expression of frataxin (FXN). However, there is still no effective treatment. Our previous studies have
Yutong Liu, Jing Cai, Jiaqi Shen, Weichen Dong, Li Xu, M. Fang, Yi-Shuan Lin, Jiali Liu, Yibing Ding, T. Qiao, Kuanyu Li   +10 more
semanticscholar   +1 more source

Copper redox chemistry of plant frataxins

Journal of Inorganic Biochemistry, 2018
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 a full study on the ability of Arabidopsis (AtFH) and Zea mays (ZmFH-1 and ZmFH-2) frataxins to assemble into disulfide-bridged ...
Sánchez, Manu, Palacios, Oscar, Buchensky, Celeste, Sabio, Laura, Gomez Casati, Diego Fabian, Pagani, María Ayelén, Capdevila, Mercè, Atrian, Silvia, Dominguez Vera, Jose M.   +8 more
openaire   +3 more sources

The kinetics of folding of frataxin

Physical Chemistry Chemical Physics, 2014
The role of the denatured state in protein folding represents a key issue for the proper evaluation of folding kinetics and mechanisms. The yeast ortholog of the human frataxin, a mitochondrial protein essential for iron homeostasis and responsible for Friedreich's ataxia, has been shown to undergo cold denaturation above 0 °C, in the absence of ...
BONETTI, DANIELA, TOTO, ANGELO, Giri R, Morrone A, Sanfelice D, Pastore A, Temussi P, GIANNI, STEFANO, BRUNORI, Maurizio   +8 more
openaire   +5 more sources

Frataxin deficiency and mitochondrial dysfunction

Mitochondrion, 2002
Friedreich ataxia (FA) is an inherited recessive disorder characterized by progressive neurological disability and heart abnormalities. The Friedreich ataxia gene (FRDA) encodes a small mitochondrial protein, frataxin, which is produced in insufficient amounts in the disease as a consequence of a GAA triplet repeat expansion in the first intron of the ...
openaire   +2 more sources

Mechanisms of iron and copper–frataxin interactions

Metallomics, 2017
Investigation of the mechanisms of mitochondrial metal binding to frataxinin vitro.
T. H. L. Han, J. M. Camadro, R. Santos, E. Lesuisse, J. M. El Hage Chahine, N. T. Ha-Duong   +5 more
openaire   +3 more sources

Correlation of frataxin content in blood and skeletal muscle endorses frataxin as a biomarker in Friedreich ataxia

Movement Disorders, 2011
AbstractBackground:Friedreich ataxia is an autosomal recessive disorder caused by mutations in the frataxin gene, leading to reduced levels of the mitochondrial protein frataxin. Assays to quantitatively measure frataxin in peripheral blood have been established.
Wolfgang, Nachbauer, Julia, Wanschitz, Hannes, Steinkellner, Andreas, Eigentler, Brigitte, Sturm, Kurt, Hufler, Barbara, Scheiber-Mojdehkar, Werner, Poewe, Markus, Reindl, Sylvia, Boesch   +9 more
openaire   +2 more sources

Candida albicans lacking the frataxin homologue: a relevant yeast model for studying the role of frataxin

Molecular Microbiology, 2004
SummaryWe cloned the CaYFH1 gene that encodes the yeast frataxin homologue in Candida albicans. CaYFH1 was expressed in Δyfh1 Saccharomyces cerevisiae cells, where it compensated for all the phenotypes tested except for the lack of cytochromes. Double ΔCayfh1/ΔCayfh1 mutant had severe defective growth, accumulated iron in their mitochondria, lacked ...
Renata, Santos, Nicole, Buisson, Simon A B, Knight, Andrew, Dancis, Jean-Michel, Camadro, Emmanuel, Lesuisse   +5 more
openaire   +2 more sources

Frataxin analysis using triple quadrupole mass spectrometry: application to a large heterogeneous clinical cohort

Journal of Neurology, 2023
David R. Lynch, Teerapat Rojsajjakul, S. Subramony, Susan Perlman, Medina Keita, C. Mesaros, Ian A. Blair   +6 more
semanticscholar   +1 more source

Halogens engineering-based design of agonists for boosting expression of frataxin protein in Friedreich's ataxia.

European Review for Medical and Pharmacological Sciences, 2023
M. Naveed, I. Ali, T. Aziz, N. Ain, M. Shabbir, K. Javed, M. Alharbi, A. Alshammari, A. F. Alasmari, S. Alharbi, M. S. Alharbi   +10 more
semanticscholar   +1 more source