Results 11 to 20 of about 10,061 (229)
The Structure and Function of Frataxin [PDF]
Critical Reviews in Biochemistry and Molecular Biology, 2006 Frataxin, a highly conserved protein found in prokaryotes and eukaryotes, is required for efficient regulation of cellular iron homeostasis. Humans with a frataxin deficiency have the cardio- and neurodegenerative disorder Friedreich’s ataxia, commonly ...
Bencze, Krisztina Z. +6 more
core +5 more sources
Thermodynamic Stabilization of Human Frataxin
bioRxiv, 2023 Recombinant proteins and antibodies are routinely used as drugs to treat prevalent diseases such as diabetes or cancer, while enzyme replacement and gene therapies are the main therapeutic intervention lines in rare diseases.
Reyes Núñez‐Franco +10 more
semanticscholar +2 more sources
Delivery of the 135kb human frataxin genomic DNA locus gives rise to different frataxin isoforms [PDF]
Genomics, 2015 © 2015 Elsevier Inc. Friedreich's ataxia (FRDA) is the most common form of hereditary ataxia caused by recessive mutations in the FXN gene. Recent results have indicated the presence of different frataxin isoforms due to alternative gene expression ...
Díaz-Nido, Javier +4 more
core +4 more sources
Identification of Frataxin as a regulator of ferroptosis
Redox Biology, 2020 Ferroptosis is a newly discovered form of non-apoptotic regulated cell death and is characterized by iron-dependent and lipid peroxidation. Due to the enhanced dependence on iron in cancer cells, induction of ferroptosis is becoming a promising ...
Jing Du +17 more
semanticscholar +4 more sources
Frataxin Loss Promotes Angiotensin II–Induced Endothelial‐to‐Mesenchymal Transition [PDF]
Journal of the American Heart Association: Cardiovascular and Cerebrovascular DiseaseBackground The metabolic flexibility of endothelial cells is linked to their phenotypic plasticity. Frataxin is critical in determining the iron metabolism and fate of endothelial cells.
Yuetong Guo +4 more
doaj +2 more sources
Hypomagnetic Fields Influence the Developmental Duration, Fecundity and Temperature Stress Resistance of Drosophila melanogaster via Frataxin-Associated Traits [PDF]
BiologyFrataxin is a highly conserved mitochondrial protein that plays a key role in iron homeostasis and metabolism, and its deficiency leads to oxidative stress, mitochondrial dysfunction, and neurodegeneration.
Huiming Kang +3 more
doaj +2 more sources
Journal of Biological Chemistry, 2022 Friedreich’s ataxia (FRDA) is a degenerative disease caused by a decrease in the mitochondrial protein frataxin (Fxn), which is involved in iron–sulfur cluster (ISC) synthesis. Diminutions in Fxn result in decreased ISC synthesis, increased mitochondrial
P. Hackett, X. Jia, Liangtao Li, D. Ward
semanticscholar +3 more sources
Human Frataxin: Iron and Ferrochelatase Binding Surface [PDF]
Chem. Commun., 2007 The coordinated iron structure and ferrochelatase binding surface of human frataxin have been characterized to provide insight into the protein’s ability to serve as the iron chaperone during heme ...
Bencze, Krisztina Z. +6 more
core +5 more sources
Redox Biology, 2020 Friedreich ataxia (FA) is a cardioneurodegenerative disease caused by deficient frataxin expression. This mitochondrial protein has been related to iron homeostasis, energy metabolism, and oxidative stress.
Rosa Purroy +3 more
doaj +2 more sources
Pharmacological screening using an FXN-EGFP cellular genomic reporter assay for the therapy of Friedreich ataxia. [PDF]
PLoS ONE, 2013 Friedreich ataxia (FRDA) is an autosomal recessive disorder characterized by neurodegeneration and cardiomyopathy. The presence of a GAA trinucleotide repeat expansion in the first intron of the FXN gene results in the inhibition of gene expression and ...
Lingli Li +6 more
doaj +4 more sources