Results 51 to 60 of about 389 (134)

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, Santos Renata, Bayot Aurélien, Rustin Pierre   +3 more
doaj   +1 more source

Exploring and Targeting the Connection of Iron and Copper Homeostasis to Neurodegenerative Diseases

MedComm, Volume 7, Issue 6, June 2026.
Iron and copper dyshomeostasis, along with their interactions with key intrinsically disordered proteins (e.g., Aβ, tau, α‐synuclein) have a strong implication in the onset and progression of neurodegenerative diseases, including Alzheimer's disease (AD), Parkinson's disease (PD), Prion diseases (PrDs), Huntington's disease (HD), Wilson's disease (WD),
Xin Liu, Lin Jia, Kerong Wu, Mo Chen, Jichao Sun, Chuanbin Yang, Chengchao Xu, Jie Sun, Jigang Wang, Lingyun Dai   +9 more
wiley   +1 more source

Pathological frataxin deficiency in mice causes tissue-specific alterations in iron homeostasis

iScience
Summary: Friedreich ataxia is caused by partial frataxin deficiency due to genetic mutations. It is well established that frataxin knockout affects iron homeostasis, but the alterations caused by pathological (partial) frataxin deficiency are poorly ...
Maria Pazos-Gil, Marta Medina-Carbonero, Arabela Sanz-Alcázar, Marta Portillo-Carrasquer, Luiza Oliveira-Jorge, Gonzalo Hernández, Mayka Sánchez, Fabien Delaspre, Elisa Cabiscol, Joaquim Ros, Jordi Tamarit   +10 more
doaj   +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

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, Simon A B Knight, Alok Pandey, Jayashree Pain, Serdar Turkarslan, Debkumar Pain, Andrew Dancis   +6 more
doaj   +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

Nomlabofusp Treatment Produces Frataxin Levels That Correlate Across Peripheral Tissues: Preclinical and Clinical Support for Surrogate Tissue Sampling

Clinical and Translational Science, Volume 19, Issue 5, May 2026.
ABSTRACT Nomlabofusp is a recombinant, cell‐penetrating human frataxin (hFXN) fusion protein in development for the treatment of Friedreich's ataxia (FRDA). This study evaluated whether nomlabofusp‐derived hFXN concentrations covary across accessible peripheral matrices and FRDA‐relevant tissues, supporting the feasibility of surrogate tissue sampling ...
Flavia De Toni, Mohamed Hamdani, Chintan Patadia, Gopi Shankar   +3 more
wiley   +1 more source

Chemical shift assignment of a thermophile frataxin [PDF]

Biomolecular NMR Assignments, 2017
Frataxin is the protein responsible for the genetically-inherited neurodegenerative disease Friedreich's ataxia caused by partial silencing of the protein and loss of function. Although the frataxin function is not yet entirely clear, it has been associated to the machine that builds iron-sulfur clusters, essential prosthetic groups involved in several
Rasheed M, Yan R, Kelly G, Pastore A
openaire   +4 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

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