Results 141 to 150 of about 3,918 (176)

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 therapeutic strategy.
Jing Du, Yanchun Li, Xueying Ren
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Frataxin fracas

Nature Genetics, 1997
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Cossee M, Campuzano V, Koutnikova H, Fischbeck K, Mandel JL, Koenig M, Bidichandani SI, Patel PI, Molte MD, Canizares J, DeFrutos R, Pianese L, Cavalcanti F, MONTICELLI A, Cocozza S, Montermini L, Pandolfo M   +16 more
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Frataxin and Mitochondrial FeS Cluster Biogenesis [PDF]

Journal of Biological Chemistry, 2010
Friedreich ataxia is an inherited neurodegenerative disease caused by frataxin deficiency. Frataxin is a conserved mitochondrial protein that plays a role in FeS cluster assembly in mitochondria. FeS clusters are modular cofactors that perform essential functions throughout the cell.
Timothy L Stemmler, Emmanuel Lesuisse, Debkumar Pain   +2 more
exaly   +3 more sources

Functional studies of frataxin

Acta Paediatrica, 2004
Mitochondria generate adenosine triphosphate (ATP) but also dangerous reactive oxygen species (ROS). One‐electron reduction of dioxygen in the early stages of the electron transport chain yields a superoxide radical that is detoxified by mitochondrial superoxide dismutase to give hydrogen peroxide.
G, Isaya, H A, O'Neill, O, Gakh, S, Park, R, Mantcheva, S M, Mooney   +5 more
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Frataxin and frataxin deficiency in Friedreich's ataxia

Journal of the Neurological Sciences, 2003
A deficiency in the protein frataxin causes Friedreich’s ataxia (FRDA). Missense mutations in f2% of disease alleles cause a functional deficiency of frataxin, while f98% of disease alleles have a guanine-adenine-adenine (GAA) repeat expansion in the first intron [1]. The expansion decreases frataxin expression by interfering with transcription [2].
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Frataxin Structure and Function

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 dynamics of frataxin, metal-ion-protein interactions, as well as on the effect of mutations on protein conformation, stability ...
Castro I. H., Pignataro M. F., Sewell K. E., Espeche L. D., Herrera M. G., Noguera M. E., Dain L., Nadra A. D., Aran M., Smal C., Gallo M., Santos J.   +11 more
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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
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Thermodynamic Stabilization of Human Frataxin

2023
ABSTRACTRecombinant 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. In protein-based therapeutics, optimizedin vivostability is key as intrinsic denaturation and intracellular proteostatic
Reyes Núñez-Franco, Angel Torres-Mozas, Claudio D. Navo, Andreas Schedlbauer, Mikel Azkargorta, Ibon Iloro, Félix Elortza, Gabriel Ortega, Oscar Millet, Francesca Peccati, Gonzalo Jiménez-Osés   +10 more
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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
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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 ...
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