Therapeutic Effect of Idebenone on Rats with Vascular Dementia via the MicroRNA-216a/RSK2/NF-κB Axis
Xudong Qian,1 Qianqian Xu,1 Guoyun Li,2 Yi Bu,1 Fan Sun,1 Jian Zhang1 1Department of Neurology, Affiliated Hospital of Chengde Medical University, Chengde, 067000, Hebei, People’s Republic of China; 2Department of Respiratory, Affiliated Hospital ...
Qian X, Xu Q, Li G, Bu Y, Sun F, Zhang J
doaj
Drosophila melanogaster как тест-объект для оценки биологического воздействия твердой фазы снега [PDF]
Several reports in the literature describe the effects of low-dose (5 mg/kg/day) idebenone in significantly reducing cardiac hypertrophy in patients with Friedreich ataxia. However, the effects of idebenone on neurological function have not been reliably
di Prospero, Nicholas A. +2 more
core +1 more source
The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues [PDF]
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.
C. Sandi +7 more
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Limb-girdle muscular dystrophy 2G (LGMD2G) is a subtype of limb-girdle muscular dystrophy. However, the disease’s mechanisms are still not fully understood, and no established therapeutic targets have been found.
Xiaoqing Lv +5 more
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The Case of a 25-Year-Old Woman With Isolated Head Tremor. [PDF]
ABSTRACT This study reported a 25‐year‐old woman with isolated head tremors as the main manifestation, along with type 1 diabetes, bilateral hearing loss, and leukoencephalopathy, who was diagnosed with mitochondrial disease due to a single large mtDNA deletion (m.8647‐16082del).
Zhao Y +5 more
europepmc +2 more sources
In Vitro Antioxidant Activity and In Vivo Topical Efficacy of Lipid Nanoparticles Co-Loading Idebenone and Tocopheryl Acetate [PDF]
Idebenone (IDE) is a strong antioxidant that has been proposed for the treatment of skin disorders, including skin ageing. Unfavorable physico-chemical properties make IDE a poor skin permeant where effectiveness could be improved by its loading into ...
Arena, Rosaria +8 more
core +1 more source
Idebenone Treatment In Leber's Hereditary Optic Neuropathy [PDF]
Sir, We have read with great interest the results presented by Klopstock et al. (2011) concerning the RHODOS study on a clinical trial with idebenone in Leber's hereditary optic neuropathy (LHON) and we would like to share our own experience of idebenone therapy in LHON.
CARELLI, VALERIO +17 more
openaire +4 more sources
Oral health in two heterozygote female twins with congenital lactic acidosis [PDF]
Introduction: Congenital lactic acidosis (CLA) is a rare disease caused by mutations in Mithocondrial DNA (mtDNA), which affects the ability of cells to use energy that causes accumulation of lactic acid in the body.
GUERRA, Fabrizio +4 more
core +1 more source
Leber hereditary optic neuropathy: Current perspectives [PDF]
Leber hereditary optic neuropathy (LHON) is one of the most common inherited optic neuropathies causing bilateral central vision loss. The disorder results from point mutations in mitochondrial DNA and subsequent mitochondrial dysfunction.
McClelland, Collin +2 more
core +3 more sources
Metabolic Stability of New Mito-Protective Short-Chain Naphthoquinones
Short-chain quinones (SCQs) have been identified as potential drug candidates against mitochondrial dysfunction, which is largely dependent on their reversible redox characteristics of the active quinone core. We recently synthesized a SCQ library of >
Zikai Feng +3 more
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