Results 51 to 60 of about 5,673 (157)

Mesenchymal Stem Cell‐Based Therapy for Cerebellar Ataxia: From Bench to Bedside

CNS Neuroscience &Therapeutics, Volume 32, Issue 7, July 2026.
Allogeneic hMSCs transplanted across LPS, Ara‐C, and SCA2 cerebellar ataxia (CA) models suppress neuroinflammation and restore the neurotrophin axis, collectively preserving Purkinje cell integrity. These preclinical findings are being translated clinically, from a first‐in‐human case report to an ongoing Phase II/III randomized trial (NCT02540655 ...
Kyoungho Suk, Ho‐Won Lee, Sang Ryong Kim   +2 more
wiley   +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

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

An Exploration of Vitamin D Deficiency and Clinical Status in Friedreich's Ataxia Patients in the UK

Movement Disorders Clinical Practice, Volume 13, Issue 7, Page 1788-1789, July 2026.
Zofia Fleszar, Gilbert Thomas‐Black, Hector Garcia‐Moreno, Arron Cook, Paola Giunti   +4 more
wiley   +1 more source

Src inhibitors modulate frataxin protein levels

, 2015
Defective expression of frataxin is responsible for the inherited, progressive degenerative disease Friedreich's Ataxia (FRDA). There is currently no effective approved treatment for FRDA and patients die prematurely. Defective frataxin expression causes
Condò, I, MALISAN, FLORENCE, Fortuni, S, Moiz, S, TESTI, ROBERTO, Crescenzi, M, RUFINI, ALESSANDRA, GUCCINI, ILARIA, SERIO, DARIO, Camerini, S, Testi, R, Serio, D, Rufini, A, ARCURI, GAETANO, Guccini, I, CONDO', IVANO, Arcuri, G, Malisan, F, Cherubini, F   +18 more
core   +1 more source

Frataxin and the molecular mechanism of mitochondrial iron-loading in Friedreich's ataxia

, 2016
The mitochondrion is a major site for the metabolism of the transition metal, iron, which is necessary for metabolic processes critical for cell vitality. The enigmatic mitochondrial protein, frataxin, is known to play a significant role in both cellular
Sahni, Sumit, Lane, Darius JR, Huang, Michael L-H, Chiang, Shannon, Kovacevic, Zaklina, Richardson, Des R, Merlot, Angelica M, Kalinowski, Danuta S   +7 more
core   +1 more source

Chemical shift assignment of a thermophile frataxin [PDF]

, 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.
Geoff Kelly, Pastore, Annalisa; id_orcid, Masooma Rasheed, Pastore A, Kelly G, Kelly, Geoff, Yan R, Annalisa Pastore, Yan, Robert, Robert Yan, Rasheed M, Rasheed, Masooma   +11 more
core   +1 more source

Delivery of the 135 kb human frataxin genomic DNA locus gives rise to different frataxin isoforms

Genomics, 2015
Friedreich's ataxia (FRDA) is the most common form of hereditary ataxia caused by recessive mutations in the FXN gene. Recent results have indicated the presence of different frataxin isoforms due to alternative gene expression mechanisms. Our previous studies demonstrated the advantages of using high-capacity herpes simplex virus type 1 (HSV-1 ...
Pérez-Luz, S., Giménez-Cassina, Alfredo, Fernández-Frias, I., Wade-Martins, R., Díaz-Nido, Javier   +4 more
openaire   +3 more sources

The in vivo mitochondrial two-step maturation of human frataxin. [PDF]

, 2008
International audienceDeficiency in the nuclear-encoded mitochondrial protein frataxin causes Friedreich ataxia (FRDA), a progressive neurodegenerative disorder associating spinocerebellar ataxia and cardiomyopathy.
Stéphane Schmucker, Puccio, Hélène, Schmucker, Stéphane, Carelle-Calmels, Nadège, Martelli, Alain, Hélène Puccio, Argentini, Manuela, Nadège Carelle-Calmels, Alain Martelli, Manuela Argentini   +9 more
core   +1 more source