Results 51 to 60 of about 3,918 (176)

Frataxin, Iron–Sulfur Clusters, Heme, ROS, and Aging [PDF]

Antioxidants & Redox Signaling, 2006
A deficiency in mitochondrial frataxin causes an increased generation of mitochondrial reactive oxygen species (ROS), which may contribute to the cell degenerative features of Friedreich's ataxia. In this work the authors demonstrate mitochondrial iron-sulfur cluster (ISC) defects and mitochondrial heme defects, and suggest how both may contribute to ...
Eleonora, Napoli, Franco, Taroni, Gino A, Cortopassi   +2 more
openaire   +2 more sources

A Mussel‐Inspired Bioadhesive Patch to Selectively Kill Glioblastoma Cells

Advanced Science, Volume 13, Issue 22, 17 April 2026.
An innovative mussel‐inspired bioadhesive patch has been developed for post‐surgical glioblastoma treatment. The patch, which adheres strongly in biological environments, releases a localized treatment. This treatment, acting via reactive oxygen species, shows specific toxicity to glioblastoma cells.
Jose Bolaños‐Cardet, Sara Pugliese, Jordi Bruna, Daniel Ruiz‐Molina, Salvio Suárez‐García, Victor J. Yuste   +5 more
wiley   +1 more source

Frataxin activates mitochondrial energy conversion and oxidative phosphorylation [PDF]

Proceedings of the National Academy of Sciences, 2000
Friedreich's ataxia (FA) is an autosomal recessive disease caused by decreased expression of the mitochondrial protein frataxin. The biological function of frataxin is unclear. The homologue of frataxin in yeast, YFH1 , is required for cellular respiration and was suggested to regulate mitochondrial iron ...
M, Ristow, M F, Pfister, A J, Yee, M, Schubert, L, Michael, C Y, Zhang, K, Ueki, M D, Michael, B B, Lowell, C R, Kahn   +9 more
openaire   +2 more sources

Increased Frataxin Expression Induced in Friedreich Ataxia Cells by Platinum TALE-VP64s or Platinum TALE-SunTag

Molecular Therapy: Nucleic Acids, 2018
Frataxin gene (FXN) expression is reduced in Friedreich’s ataxia patients due to an increase in the number of GAA trinucleotides in intron 1. The frataxin protein, encoded by that gene, plays an important role in mitochondria’s iron metabolism.
Khadija Cherif, Catherine Gérard, Joël Rousseau, Dominique L. Ouellet, Pierre Chapdelaine, Jacques P. Tremblay   +5 more
doaj   +1 more source

Unraveling Chronic Pain: From Mechanisms and Risks to Diagnosis and Treatment

MedComm, Volume 7, Issue 4, April 2026.
Chronic pain arises through distinct molecular pathways categorized into nociceptive, neuropathic, and nociplastic types. Nociceptive pain begins with TRP channel activation in peripheral nociceptors, signaling via Aδ‐ and C‐fibers through the spinal dorsal horn and spinothalamic tracts to the brain, regulated by descending inhibition and involving ...
Xiaofeng Dai, Chongxiang Wang, Ping Jiang, Xiaopeng Mei   +3 more
wiley   +1 more source

Frataxin Shows Developmentally Regulated Tissue-Specific Expression in the Mouse Embryo

Neurobiology of Disease, 1997
Friedreich ataxia (FRDA) is an autosomal recessive degenerative disease caused either by an intronic GAA triplet repeat expansion that suppresses the expression of the frataxin gene on chromosome 9q13, or, rarely, by point mutations in the frataxin gene.
Sarn Jiralerspong, Yanling Liu, Laura Montermini, Stefano Stifani, Massimo Pandolfo   +4 more
doaj   +1 more source

Drug Repositioning in Friedreich Ataxia

Frontiers in Neuroscience, 2022
Friedreich ataxia is a rare neurodegenerative disorder caused by insufficient levels of the essential mitochondrial protein frataxin. It is a severely debilitating disease that significantly impacts the quality of life of affected patients and reduces ...
Alessandra Rufini, Alessandra Rufini, Alessandra Rufini, Florence Malisan, Ivano Condò, Roberto Testi, Roberto Testi   +6 more
doaj   +1 more source

Multimodal Imaging Investigation of the Dentato‐Thalamo‐Cortical Pathway in Friedreich's Ataxia

Movement Disorders, Volume 41, Issue 4, Page 909-920, April 2026.
Abstract Background Friedreich's ataxia (FRDA) is a spinocerebellar neurodegenerative disorder. The dentato‐thalamo‐cortical (DTC) pathway, an important cerebellar output involved in motor control, plays a crucial role in the neural mechanisms underlying ataxia symptoms in FRDA.
Yinghua Jing, Imis Dogan, Ravi Dadsena, Jennifer Faber, Jörg B. Schulz, Kathrin Reetz, Sandro Romanzetti, on behalf of the FACROSS study group, Stella A. Lischewski, Kerstin Konrad, Miguel Pishnamaz, Maximillian Praster, Thomas Clavel, Vera Jankowski, Joachim Jankowski, Oliver Pabst, Katharina Marx‐Schütt, Nikolaus Marx, Julia Möllmann, Malte Jacobsen, Juergen Dukart, Simon Eickhoff, Ralf‐Dieter Hilgers   +22 more
wiley   +1 more source

Frataxin levels in peripheral tissue in Friedreich ataxia [PDF]

Annals of Clinical and Translational Neurology, 2015
Friedreich ataxia (FRDA) is an autosomal recessive ataxia resulting from mutations in the frataxin gene (FXN). Such mutations, usually expanded guanine-adenine-adenine (GAA) repeats, give rise to decreased levels of frataxin protein in both affected and unaffected tissues.
Lazaropoulos, Michael, Dong, Yina, Clark, Elisia, Greeley, Nathaniel R., Seyer, Lauren A., Brigatti, Karlla W., Christie, Carlton, Perlman, Susan L., Wilmot, George R., Gomez, Christoper M., Mathews, Katherine D., Yoon, Grace, Zesiewicz, Theresa, Hoyle, Chad, Subramony, Sub H., Brocht, Alicia F., Farmer, Jennifer M., Wilson, Robert B., Deutsch, Eric C., Lynch, David R.   +19 more
openaire   +3 more sources

Dysregulation of cellular iron metabolism in Friedreich ataxia: from primary iron-sulfur cluster deficit to mitochondrial iron accumulation

Frontiers in Pharmacology, 2014
Friedreich ataxia (FRDA) is the most common recessive ataxia in the Caucasian population and is characterized by a mixed spinocerebellar and sensory ataxia frequently associating cardiomyopathy.
Alain eMartelli, Helene ePuccio
doaj   +1 more source