Results 61 to 70 of about 5,449 (210)

Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model

, 2011
NOTICE: this is the author’s version of a work that was accepted for publication in Neurobiology of Disease. Changes resulting from the publishing process, such as peer review, editing, corrections, structural formatting, and other quality control ...
Ezzatizadeh, V, Rusche, James R., Steve Jones, James R. Rusche, Gottesfeld, Joel M., Jones, S, Sandi, C, Barnes, Glenn, Joel M. Gottesfeld, Pook, MA, Pinto, RM, Al-Mahdawi, Sahar, Chiranjeevi Sandi, Sandi, Chiranjeevi, Barnes, G, Mark A. Pook, Pinto, Ricardo Mouro, Al-Mahdawi, S, Jones, Steve, Pook, Mark A., Vahid Ezzatizadeh, Ezzatizadeh, Vahid, Sahar Al-Mahdawi, Rusche, JR, Ricardo Mouro Pinto, Gottesfeld, JM, Glenn Barnes   +26 more
core   +1 more source

Coenzyme A mitigates cystine‐deprivation‐induced ferroptosis by suppressing the iron‐starvation response

The FEBS Journal, Volume 293, Issue 11, Page 3288-3302, June 2026.
Cystine (Cys2) deprivation in pancreatic cancer cells induces oxidative stress that destabilizes cytosolic iron–sulfur cluster (ISC) proteins, triggering an iron‐regulatory protein (IRP)‐mediated iron‐starvation response (ISR). This leads to increased iron uptake (via TFRC), an expanded labile iron pool, and ferroptosis.
Mingjun Tan, Yunzhan Li, Lei Sang, Min Wang, Qin Li, Zekun Li, Dongxiao Sun, Xiuchao Wang, Shengyu Yang   +8 more
wiley   +1 more source

Mapping the evolution of mitochondrial complex I through structural variation

FEBS Letters, Volume 600, Issue 10, Page 1491-1518, May 2026.
Respiratory complex I (CI) is crucial for bioenergetic metabolism in many prokaryotes and eukaryotes. It is composed of a conserved set of core subunits and additional accessory subunits that vary depending on the organism. Here, we categorize CI subunits from available structures to map the evolution of CI across eukaryotes. Respiratory complex I (CI)
Dong‐Woo Shin, Tingting Chen, James A. Letts   +2 more
wiley   +1 more source

The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues

, 2007
Friedreich ataxia (FRDA) is caused by a homozygous GAA repeat expansion mutation within intron 1 of the FXN gene, leading to reduced expression of frataxin protein.
Pook, MA, Mouro Pinto, R, Al-Mahdawi, S, Trabzuni, D, Sandi, C   +4 more
core   +1 more source

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

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

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

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

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