Novel frataxin isoforms may contribute to the pathological mechanism of friedreich ataxia [PDF]
, 2012 This is an open-access article, free of all copyright, and may be freely reproduced, distributed, transmitted, modified, built upon, or otherwise used by anyone for any lawful purpose.
Di Zhou +11 more
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The effects of idebenone on mitochondrial bioenergetics
Biochimica et Biophysica Acta (BBA) - Bioenergetics, 2012 We have studied the effects of idebenone on mitochondrial function in cybrids derived from one normal donor (HQB17) and one patient harboring the G3460A/MT-ND1 mutation of Leber's Hereditary Optic Neuropathy (RJ206); and in XTC.UC1 cells bearing a premature stop codon at amino acid 101 of MT-ND1 that hampers complex I assembly. Addition of idebenone to
Giorgio V +6 more
openaire +5 more sources
In Vitro Antioxidant Activity of Idebenone Derivative-Loaded Solid Lipid Nanoparticles
Molecules, 2017 Idebenone (IDE) has been proposed for the treatment of neurodegenerative diseases involving mitochondria dysfunctions. Unfortunately, to date, IDE therapeutic treatments have not been as successful as expected.
Lucia Montenegro +6 more
doaj +1 more source
Effects of Idebenone on Rat Schwann Cells with Toxicity Induced by Hydrogen Peroxide: Assessment of Molecular, Apoptosis, and Oxidative Stress Parameters [PDF]
Cell JournalObjective: Schwann cells are the main cells for myelination and regeneration of peripheral nerves. Idebenone is asynthetic antioxidant used to treat central nervous system diseases.
Sam Zarbakhsh, Parisa Hayat
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Protective activity of aromatic amines and imines against oxidative nerve cell death [PDF]
, 2001 Oxidative stress is a widespread phenomenon in the pathology of neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, and amyotrophic lateral sclerosis.
Behl, Christian +3 more
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Brain and Behavior, Volume 15, Issue 11, November 2025.We report a 32‐year‐old female patient with SANDO syndrome (a subtype of mitochondrial encephalomyopathy), who carries two heterozygous POLG mutations: a novel c.3297G>C and a known c.1774C>T. The neuronal models expressing these mutations demonstrated mitochondrial dysfunction, thereby confirming their pathogenicity.
Fanjing Zhou +6 more
wiley +1 more source
Does modulation of the endocannabinoid system have potential therapeutic utility in cerebellar ataxia? [PDF]
, 2016 Cerebellar ataxias represent a spectrum of disorders which are, however, linked by common symptoms of motor incoordination and are typically associated with deficient in Purkinje cell firing activity and, often, degeneration. Cerebellar ataxias currently
Stephens, G. J.
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CPT: Pharmacometrics &Systems Pharmacology, Volume 14, Issue 11, Page 1787-1796, November 2025.Abstract Duchenne muscular dystrophy (DMD), a rare pediatric disease, presents numerous challenges when designing clinical trials, mainly due to the scarcity of available trial participants and the heterogeneity of disease progression. A quantitative clinical trial simulator (CTS) has been developed based on previously published five disease ...
Jordan Wilk +18 more
wiley +1 more source
Cardiovascular mitochondrial dysfunction induced by cocaine: biomarkers and possible beneficial effects of modulators of oxidative stress [PDF]
, 2017 Cocaine abuse has long been known to cause morbidity and mortality due to its cardiovascular toxic effects. The pathogenesis of the cardiovascular toxicity of cocaine use has been largely reviewed, and the most recent data indicate a fundamental role of ...
Arese, Marzia +5 more
core +3 more sources
Unrecognized preanalytical problem with the spectrophotometric analysis of cerebrospinal fluid for xanthochromia [PDF]
, 2008 Udgivelsesdato: 2008 ...
Hansen, Annebirthe Bo +3 more
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