Results 91 to 100 of about 5,449 (210)

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, Krisztina Z, Bencze, Ana D, Jankovic, Anna K, Crater, Courtney N, Busch, Patrick B, Bradley, Ann J, Stemmler, Mark R, Spaller, Timothy L, Stemmler   +8 more
openaire   +2 more sources

High‐Throughput Platform for Discovery of Chemical Inhibitors of Heat Shock Protein 70 (Hsp70): Adaptation for the Specialized Bacterial HscA–HscB–IscU Complex

Chemical Biology &Drug Design, Volume 107, Issue 3, March 2026.
The HscA–HscB–IscU system functions in iron–sulfur (Fe‐S) cluster biogenesis in bacteria and fungi, including pathogenic strains. Here, we describe a high‐throughput screen (HTS) to measure inorganic phosphate release from the ATPase HscA within this reconstituted, multi‐protein complex.
Arielle Shkedi, Aneta Grabinska‐Rogala, Jason Hernandez, Rafał Dutkiewicz, Jaroslaw Marszalek, Jason E. Gestwicki   +5 more
wiley   +1 more source

ALDH2 Mediated Ferroptosis Regulation in Ischemia–Reperfusion Injury

Journal of Cellular and Molecular Medicine, Volume 30, Issue 6, March 2026.
ABSTRACT Ischemia–reperfusion injury (IRI) is a common complication in diverse clinical settings, including myocardial infarction, stroke, organ transplantation and major surgery. Its pathological core lies in the metabolic disruption caused by blood flow cessation, followed by oxidative stress, inflammatory cascades and cell death upon reperfusion ...
Liang Han, Wen Zhai
wiley   +1 more source

Ancestral roles of eukaryotic frataxin: mitochondrial frataxin function and heterologous expression of hydrogenosomal Trichomonas homologs in trypanosomes.

, 2008
Frataxin is a small conserved mitochondrial protein; in humans, mutations affecting frataxin expression or function result in Friedreich's ataxia. Much of the current understanding of frataxin function comes from informative studies with yeast models ...
Lukes, Julius, Des Richardson, Julius Lukeš, Ginger, Michael L., Jan Mach, Mach, Jan, Shaojun Long, Milan Jirků, Robert Sutak, Jirku, Milan, Richardson, Des, Tachezy, Jan, Michael L. Ginger, Jan Tachezy, Long, Shaojun, Sutak, Robert   +15 more
core   +1 more source

Placental iron utilisation in fetal growth restriction: alterations in mitochondrial haem synthesis and iron–sulphur cluster assembly pathways

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, Heather C. Murray, Siddharth Acharya, Kirsty G. Pringle, Roger Smith, Joshua J. Fisher   +5 more
wiley   +1 more source

Adding a temporal dimension to the study of Friedreich's ataxia: the effect of frataxin overexpression in a human cell model

Disease Models & Mechanisms, 2018
The neurodegenerative disease Friedreich's ataxia is caused by lower than normal levels of frataxin, an important protein involved in iron–sulfur (Fe-S) cluster biogenesis.
Tommaso Vannocci, Roberto Notario Manzano, Ombretta Beccalli, Barbara Bettegazzi, Fabio Grohovaz, Gianfelice Cinque, Antonio de Riso, Luca Quaroni, Franca Codazzi, Annalisa Pastore   +9 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, Jennifer M. Farmer, David R. Lynch   +2 more
wiley   +1 more source

Frataxin knockdown in human astrocytes triggers cell death and the release of factors that cause neuronal toxicity

Neurobiology of Disease, 2015
Friedreich's ataxia (FA) is a recessive, predominantly neurodegenerative disorder caused in most cases by mutations in the first intron of the frataxin (FXN) gene.
Frida Loría, Javier Díaz-Nido
doaj   +1 more source

Detection of frataxin-EGFP fusion protein.

, 2014
Western blot analysis was carried out on protein extracts from liver tissue of FXN-EGFP transgenic mice and C57BL/6 control mice. Identical blots were probed with either anti-human frataxin monoclonal antibody (A) or with anti-GFP polyclonal antibody (B).
David I. Finkelstein (227752), Lingli Li (61106), Timothy P. Holloway (542599), Panos A. Ioannou (214510), Joseph P. Sarsero (214516)   +4 more
core   +1 more source

CRISPR/Cas9-based edition of frataxin gene in Dictyostelium discoideum

, 2023
In this paper, we describe the development of a Dictyostelium discoideum strain deficient in frataxin protein (FXN). We investigated the conservation of function between humans and D.
Velázquez Duarte, Francisco, Ibañez, Lorena Itatí, Pavan, Maria Florencia, Olmos, Justo, Pignataro, María Florencia, Santos, Javier, Gentili, Hernan Gustavo   +6 more
core   +1 more source