Results 111 to 120 of about 10,061 (229)
Frataxins Emerge as New Players of the Intracellular Antioxidant Machinery
Antioxidants, 2021 Frataxin is a mitochondrial protein which deficiency causes Friedreich’s ataxia, a cardio-neurodegenerative disease. The lack of frataxin induces the dysregulation of mitochondrial iron homeostasis and oxidative stress, which finally causes the neuronal ...
Ana Belén Uceda +4 more
doaj +1 more source
Frataxin and endothelial cell senescence in pulmonary hypertension.
Journal of Clinical Investigation, 2021 Pulmonary hypertension (PH), increased blood pressure within the lungs, is classified into five diagnostic groups based on etiology, with treatment assigned on this basis.
A. Lawrie, S. Francis
semanticscholar +1 more source
P53 binds preferentially to non-B DNA structures formed by the pyrimidine-rich strands of GaA·TTC trinucleotide repeats associated with Friedreich’s ataxia [PDF]
, 2019 Expansions of trinucleotide repeats (TNRs) are associated with genetic disorders such as Friedreich’s ataxia. The tumor suppressor p53 is a central regulator of cell fate in response to different types of insults.
Adámik, Matej +11 more
core +1 more source
The Journal of Physiology, Volume 604, Issue 5, Page 2229-2249, 1 March 2026.Abstract figure legend Altered iron handling and mitochondrial pathways in FGR placentas. Placental tissue from FGR pregnancies showed increased expression of iron importers, transferrin and divalent metal transporter 1, and decreased expression of the iron exporter ferroportin, suggesting that the FGR placenta is retaining iron to meet its own ...
Veronica B. Botha +5 more
wiley +1 more source
Friedreich's ataxia: the vicious circle hypothesis revisited
BMC Medicine, 2011 Friedreich's ataxia, the most frequent progressive autosomal recessive disorder involving the central and peripheral nervous systems, is mostly associated with unstable expansion of GAA trinucleotide repeats in the first intron of the FXN gene, which ...
Camadro Jean-Michel +3 more
doaj +1 more source
Domain Specific Placebo Response in the Modified Friedreich's Ataxia Rating Scale
Annals of Clinical and Translational Neurology, Volume 13, Issue 2, Page 393-398, February 2026.ABSTRACT The placebo response in clinical trials in ataxias complicates outcome interpretation and potentially obscures genuine treatment effects. We analyzed placebo group data from past trials in Friedreich Ataxia and observed notable responses in appendicular items, in contrast to minimal changes in axial function, as measured by respective ...
Christian Rummey +2 more
wiley +1 more source
Cisplatin May Induce Frataxin Expression
Journal of Nippon Medical School, 2003 Cisplatin is a widely used drug in cancer chemotherapy and resistance to cisplatin is a major limitation for its successful application. Intracellular inactivation of cisplatin and detoxification of reactive oxygen species (ROS) by glutathione (a crucial cellular antioxidant) is a mechanism for cisplatin resistance.
openaire +3 more sources
Monomeric Yeast Frataxin Is an Iron-Binding Protein [PDF]
Biochemistry, 2006 Friedreich's ataxia, an autosomal cardio- and neurodegenerative disorder that affects 1 in 50,000 humans, is caused by decreased levels of the protein frataxin. Although frataxin is nuclear-encoded, it is targeted to the mitochondrial matrix and necessary for proper regulation of cellular iron homeostasis.
Jeremy D, Cook +8 more
openaire +2 more sources
Turning Saccharomyces cerevisiae into a Frataxin-Independent Organism.
PLoS Genetics, 2015 Frataxin (Yfh1 in yeast) is a conserved protein and deficiency leads to the neurodegenerative disease Friedreich's ataxia. Frataxin is a critical protein for Fe-S cluster assembly in mitochondria, interacting with other components of the Fe-S cluster ...
Heeyong Yoon +6 more
doaj +1 more source
The N-terminus of mature human frataxin is intrinsically unfolded [PDF]
, 2009 Frataxin is a highly conserved nuclear-encoded mitochondrial protein whose deficiency is the primary cause of Friedreich's ataxia, an autosomal recessive neurodegenerative disease.
Adamec +33 more
core +1 more source