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Idebenone

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https://www.readbyqxmd.com/read/29857987/effect-of-the-antioxidant-idebenone-on-maternal-diabetes-induced-embryo-alterations-during-early-organogenesis
#1
Romina Higa, Sabrina Roberti, María Belén Mazzucco, Verónica White, Alicia Jawerbaum
RESEARCH QUESTION: Can maternal treatments with idebenone, a structural analogue of coenzyme Q10, prevent alterations on markers of proinflammatory-prooxidant processes, on the expression of genes involved in mitochondrial biogenesis and function, and on the apoptotic rate in embryos from mild diabetic rats? DESIGN: A mild diabetic rat model was induced by neonatal-streptozotocin administration (90 mg/kg subcutaneously). Female diabetic rats and controls were mated with healthy males...
May 21, 2018: Reproductive Biomedicine Online
https://www.readbyqxmd.com/read/29796436/leber-s-hereditary-optic-neuropathy-case-report
#2
Raluca Eugenia Iorga, Ruxandra Mihailovici, Manuela Ramona Ozturk, Dănuţ Costin
Leber's hereditary optic neuropathy is the most common mitochondrial condition and is characterized by bilateral, painless, subacute visual loss that develops during young adult life. LHON is a rare condition and this lack of knowledge can make doctors suspect and treat for other causes of vision loss. Typically, a series of tests are performed to confirm LHON diagnosis or exclude any other conditions. We presented the case of two brothers, HB, of 40 years old and HF, of 38 years old, who presented with a decrease in visual acuity in both eyes...
January 2018: Romanian Journal of Ophthalmology
https://www.readbyqxmd.com/read/29746824/reduction-of-2-methoxy-1-4-naphtoquinone-by-mitochondrially-localized-nqo1-yielding-nad-supports-substrate-level-phosphorylation-during-respiratory-inhibition
#3
Dora Ravasz, Gergely Kacso, Viktoria Fodor, Kata Horvath, Vera Adam-Vizi, Christos Chinopoulos
Provision of NAD+ for oxidative decarboxylation of alpha-ketoglutarate to succinyl-CoA by the ketoglutarate dehydrogenase complex (KGDHC) is critical for maintained operation of succinyl-CoA ligase yielding high-energy phosphates, a process known as mitochondrial substrate-level phosphorylation (mSLP). We have shown previously that when NADH oxidation by complex I is inhibited by rotenone or anoxia, mitochondrial diaphorases yield NAD+ , provided that suitable quinones are present (Kiss G et al., FASEB J 2014, 28:1682)...
May 7, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29694828/the-idebenone-metabolite-qs10-restores-electron-transfer-in-complex-i-and-coenzyme-q-defects
#4
REVIEW
Valentina Giorgio, Marco Schiavone, Chiara Galber, Marco Carini, Tatiana Da Ros, Valeria Petronilli, Francesco Argenton, Valerio Carelli, Manuel J Acosta Lopez, Leonardo Salviati, Maurizio Prato, Paolo Bernardi
No abstract text is available yet for this article.
April 22, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29587845/patient-derived-lymphoblastoid-cell-lines-harboring-mitochondrial-dna-mutations-as-tool-for-small-molecule-drug-discovery
#5
Randall Marcelo Chin, Tadas Panavas, Jeffrey M Brown, Krista K Johnson
OBJECTIVE: Mitochondrial diseases are a group of devastating disorders for which there is no transformative cure. The majority of therapies for mitochondrial disease-approved, previously tested, or currently in development-are small molecules. The implementation of better cell-based models of mitochondrial disease can accelerate and improve the accuracy of small molecule drug discovery. The objective of this study is to evaluate the use of patient-derived lymphoblastoid cell lines for small molecule research in mitochondrial disease...
March 27, 2018: BMC Research Notes
https://www.readbyqxmd.com/read/29552298/synergistic-neuroprotective-effect-of-rasagiline-and-idebenone-against-retinal-ischemia-reperfusion-injury-via-the-lin28-let-7-dicer-pathway
#6
Dawei Lei, Zhengbo Shao, Xinrong Zhou, Huiping Yuan
Retinal ischemia-reperfusion (RIR) injury causes neuronal degeneration and initiates various optic nerve diseases. This study aimed to investigate the synergistic neuroprotective effect of rasagiline and idebenone against RIR injury. A combination of rasagiline and idebenone was administered intraperitoneally immediately after establishment of the RIR model. Treatment with the combination of the two drugs resulted in a significant restoration of retinal thickness and retinal ganglion cells. Apoptosis of cells in ganglion cell layers was also ameliorated, suggesting that the effect of the two drugs was synergistic and the expression of brain-derived neurotrophic factor increased...
February 23, 2018: Oncotarget
https://www.readbyqxmd.com/read/29490390/-hereditary-optic-neuropathies
#7
Klaus Rüther
Hereditary optic nerve disorders are rare. For ophthalmologists, Leber's hereditary optic neuropathy (LHON) and autosomal dominant optic atrophy (ADOA) are of particular relevance. LHON and ADOA are diseases of the retinal ganglion cells and are caused by mitchochondrial dysfunction. LHON is based on mutations of the mitochondrial, ADOA of the nuclear DNA. LHON is a disease that usually leads to severe visual impairment (visual acuity < 0.1). Since there is an approved therapy for LHON (Idebenone [Raxone]), the diagnosis has to be confirmed immediately by means of molecular genetic diagnostics...
February 28, 2018: Klinische Monatsblätter Für Augenheilkunde
https://www.readbyqxmd.com/read/29467808/sibling-ethambutol-optic-chiasmopathy
#8
Viran Jayanetti, Michael Rossiter-Thornton, Domit Azar, Clare L Fraser
Ethambutol is utilised in the treatment of Mycobacterium avium and Mycobacterium tuberculosis infection. The authors report two siblings who developed the adverse effect of ethambutol-induced optic chiasmopathy, with recovery following cessation of ethambutol. Discussion explores potential genetic predisposition to development of this condition and its resolution. Ethambutol optic neuropathy (EON), Leber's hereditary optic neuropathy (LHON), and other optic neuropathies of mitochondrial origin share a common pathophysiology...
February 2018: Neuro-ophthalmology
https://www.readbyqxmd.com/read/29401722/idebenone-novel-strategies-to-improve-its-systemic-and-local-efficacy
#9
REVIEW
Lucia Montenegro, Rita Turnaturi, Carmela Parenti, Lorella Pasquinucci
The key role of antioxidants in treating and preventing many systemic and topical diseases is well recognized. One of the most potent antioxidants available for pharmaceutical and cosmetic use is Idebenone (IDE), a synthetic analogue of Coenzyme Q10 . Unfortunately, IDE's unfavorable physicochemical properties such as poor water solubility and high lipophilicity impair its bioavailability after oral and topical administration and prevent its parenteral use. In recent decades, many strategies have been proposed to improve IDE effectiveness in the treatment of neurodegenerative diseases and skin disorders...
February 5, 2018: Nanomaterials
https://www.readbyqxmd.com/read/29393352/effect-of-idebenone-on-bone-marrow-mesenchymal-stem-cells-in-vitro
#10
Jiahui Zhang, Jun Zhang, Tao Li, Nannan Zhang, Shengnan Tang, Zehua Tao, Xiaohe Zhou, Xiaochun Sun, Huabiao Chen
In recent years, stem cell research has continued to benefit from its crossover with chemistry, particularly the investigation of small molecular drugs modulating specific targets to regulate stem cell fate. Idebenone (IDB) is a yellow crystalline powder that is used in the treatment of chronic cerebrovascular diseases. The objective of the present study was to examine whether IDB had an influence on bone marrow‑derived mesenchymal stem cells (BMSCs) extracted from the bone marrow of Sprague‑Dawley rats...
April 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29210930/leber-hereditary-optic-neuropathy-caused-by-a-mitochondrial-dna-10663t-c-point-mutation-and-its-response-to-idebenone-treatment
#11
Øystein Kalsnes Jørstad, Eva Meling Ødegaard, Ketil Riddervold Heimdal, Emilia Kerty, Ketil Riddervold Heimdal, Emilia Kerty
No abstract text is available yet for this article.
March 2018: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/29133631/mitochondrial-genetics-and-therapeutic-overview-of-leber-s-hereditary-optic-neuropathy
#12
REVIEW
Agaath Hedina Manickam, Minu Jenifer Michael, Sivasamy Ramasamy
Leber's hereditary optic neuropathy (LHON) is a common inherited mitochondrial disorder that is characterized by the degeneration of the optic nerves, leading to vision loss. The major mutations in the mitochondrial genes ND1, ND4, and ND6 of LHON subjects are found to increase the oxidative stress experienced by the optic nerve cell, thereby leading to nerve cell damage. Accurate treatments are not available and drugs that are commercially available like Idebenone, EPI-743, and Bendavia with their antioxidant role help in reducing the oxidative stress experienced by the cell thereby preventing the progression of the disease...
November 2017: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/29054473/mitochondrial-disorders-of-the-retinal-ganglion-cells-and-the-optic-nerve
#13
REVIEW
Josef Finsterer, Michelangelo Mancuso, Davide Pareyson, Jean-Marc Burgunder, Thomas Klopstock
OBJECTIVES: To summarise and discuss recent findings and future perspectives concerning mitochondrial disorders (MIDs) affecting the retinal ganglion cells and the optic nerve (mitochondrial optic neuropathy. MON). METHOD: Literature review. RESULTS: MON in MIDs is more frequent than usually anticipated. MON may occur in specific as well as non-specific MIDs. In specific and non-specific MIDs, MON may be a prominent or non-prominent phenotypic feature and due to mutations in genes located either in the mitochondrial DNA (mtDNA) or the nuclear DNA (nDNA)...
October 18, 2017: Mitochondrion
https://www.readbyqxmd.com/read/29047345/visual-prognosis-better-in-eyes-with-less-severe-reduction-of-visual-acuity-one-year-after-onset-of-leber-hereditary-optic-neuropathy-caused-by-the-11-778-mutation
#14
Yukihiko Mashima, Kazuteru Kigasawa, Kei Shinoda, Masato Wakakura, Yoshihisa Oguchi
BACKGROUND: Patients with Leber hereditary optic neuropathy (LHON) have a progressive decrease of their visual acuity which can deteriorate to <0.1. Some patients can have a partial recovery of their vision in one or both eyes. One prognostic factor associated with a recovery of vision is an early-age onset. The purpose of this study was to determine other clinical factors that are predictive of a good visual recovery. METHODS: Sixty-one Japanese LHON patients, with the 11,778 mutation and a mean age of 23...
October 18, 2017: BMC Ophthalmology
https://www.readbyqxmd.com/read/28991104/international-consensus-statement-on-the-clinical-and-therapeutic-management-of-leber-hereditary-optic-neuropathy
#15
Valerio Carelli, Michele Carbonelli, Irenaeus F de Coo, Aki Kawasaki, Thomas Klopstock, Wolf A Lagrèze, Chiara La Morgia, Nancy J Newman, Christophe Orssaud, Jan Willem R Pott, Alfredo A Sadun, Judith van Everdingen, Catherine Vignal-Clermont, Marcela Votruba, Patrick Yu-Wai-Man, Piero Barboni
Leber hereditary optic neuropathy (LHON) is currently estimated as the most frequent mitochondrial disease (1 in 27,000-45,000). Its molecular pathogenesis and natural history is now fairly well understood. LHON also is the first mitochondrial disease for which a treatment has been approved (idebenone-Raxone, Santhera Pharmaceuticals) by the European Medicine Agency, under exceptional circumstances because of the rarity and severity of the disease. However, what remains unclear includes the optimal target population, timing, dose, and frequency of administration of idebenone in LHON due to lack of accepted definitions, criteria, and general guidelines for the clinical management of LHON...
December 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/28943110/therapies-for-mitochondrial-diseases-and-current-clinical-trials
#16
REVIEW
Ayman W El-Hattab, Ana Maria Zarante, Mohammed Almannai, Fernando Scaglia
Mitochondrial diseases are a clinically and genetically heterogeneous group of disorders that result from dysfunction of the mitochondrial oxidative phosphorylation due to molecular defects in genes encoding mitochondrial proteins. Despite the advances in molecular and biochemical methodologies leading to better understanding of the etiology and mechanism of these diseases, there are still no satisfactory therapies available for mitochondrial disorders. Treatment for mitochondrial diseases remains largely symptomatic and does not significantly alter the course of the disease...
November 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28869486/efficacy-of-idebenone-to-preserve-respiratory-function-above-clinically-meaningful-thresholds-for-forced-vital-capacity-fvc-in-patients-with-duchenne-muscular-dystrophy
#17
MULTICENTER STUDY
Oscar H Mayer, Mika Leinonen, Christian Rummey, Thomas Meier, Gunnar M Buyse
BACKGROUND: Patients with DMD experience progressive restrictive respiratory disease and eventual respiratory failure. Standard of care guidelines command changes in disease management when forced vital capacity percent of predicted (FVC% p) falls below clinically relevant thresholds. The Phase 3 DELOS trial in patients with DMD demonstrated that idebenone reduces the loss of peak expiratory flow and FVC compared to placebo (Buyse GM, et al.; Lancet 2015; 385 : 1748-57). OBJECTIVE: Post-hoc analyses were conducted to assess whether treatment with idebenone could reduce the risk of patients dropping below clinically meaningful thresholds of FVC% p...
2017: Journal of Neuromuscular Diseases
https://www.readbyqxmd.com/read/28699051/-assessing-the-added-benefit-of-new-ophthalmic-drugs-which-additional-insights-can-be-extracted-from-the-early-benefit-assessment
#18
REVIEW
M Appelrath, G Glaeske
Until now six ophthalmic agents have undergone the German early benefit assessment according to § 35a Social Security Code (SGB) V in a total of eleven indications. Only one agent (ocriplasmin) was recognized by the Federal Joint Committee as having an added benefit based on submitted study data for a subpopulation (indication of a considerable added benefit, limited for 5 years) and another agent, idebenone, received an added benefit due to its orphan drug designation (proof of a not quantifiable added benefit, limited for 2 years)...
December 2017: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
https://www.readbyqxmd.com/read/28648635/pro-oxidant-pro-inflammatory-alterations-in-the-offspring%C3%A2-s-heart-of-mild-diabetic-rats-are-regulated-by-maternal-treatments-with-a-mitochondrial-antioxidant
#19
Romina Higa, Sabrina L Roberti, Evangelina Capobianco, Daiana Fornes, Verónica White, Alicia Jawerbaum
Maternal diabetes programs metabolic and cardiovascular diseases in the offspring. Here, we demonstrated increased pro-oxidant/pro-inflammatory markers in the heart of 2-day-old offspring of diabetic rats, previous to the induction of metabolic alterations. At a pre-pubertal stage, sex-dependent changes were evidenced in the diabetic group, as only males showed increased glycemia as well as increased concentrations of nitrated proteins, matrix metalloproteinase-9 and peroxisome proliferator activated receptor α (PPARα) in the heart...
June 23, 2017: Reproductive Toxicology
https://www.readbyqxmd.com/read/28643190/mitochondrial-biogenesis-and-neural-differentiation-of-human-ipsc-is-modulated-by-idebenone-in-a-developmental-stage-dependent-manner
#20
J Augustyniak, J Lenart, M Zychowicz, P P Stepien, L Buzanska
Idebenone, the synthetic analog of coenzyme Q10 can improve electron transport in mitochondria. Therefore, it is used in the treatment of Alzheimer's disease and other cognitive impairments. However, the mechanism of its action on neurodevelopment is still to be elucidated. Here we demonstrate that the cellular response of human induced pluripotent stem cells (hiPSC) to idebenone depends on the stage of neural differentiation. When: neural stem cells (NSC), early neural progenitors (eNP) and advanced neural progenitors (NP) have been studied a significant stimulation of mitochondrial biogenesis was observed only at the eNP stage of development...
August 2017: Biogerontology
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