coenzyme-q10 and Retinal-Diseases

Ocular neurodegenerative diseases such as glaucoma, diabetic retinopathy, and age-related macular degeneration are common retinal diseases responsible for most of the blindness causes in the working-age and elderly populations in developed countries. Many of the current treatments used in these pathologies fail to stop or slow the progression of the disease. Therefore, other types of treatments with neuroprotective characteristics may be necessary to allow a more satisfactory management of the disease. Citicoline and coenzyme Q10 are molecules that have neuroprotective, antioxidant, and anti-inflammatory properties, and their use could have a beneficial effect in ocular neurodegenerative pathologies. This review provides a compilation, mainly from the last 10 years, of the main studies that have been published on the use of these drugs in these neurodegenerative diseases of the retina, analyzing the usefulness of these drugs in these pathologies.

Topics: Aged; Cytidine Diphosphate Choline; Humans; Neurodegenerative Diseases; Retina; Retinal Diseases

Coenzyme Q10 (CoQ10) plays a critical role in mitochondrial oxidative phosphorylation by serving as an electron carrier in the respiratory electron transport chain. CoQ10 also functions as a lipid-soluble antioxidant by protecting lipids, proteins and DNA damaged by oxidative stress. CoQ10 deficiency has been associated with a number of human diseases in which CoQ10 supplementation therapy has been effective in slowing or reversing pathological changes. Oxidative stress is a major contributory factor in the process of retinal degeneration.. The related literature was reviewed through searching PubMed using keywords: CoQ10, CoQ10 and oxidative stress, CoQ10 and retinal degeneration. The functions of CoQ10 were summarized and its use in the treatment of age-related macular degeneration and glaucoma highlighted. The therapeutic potential of CoQ10 for other retinal diseases was also discussed.. CoQ10 has been applied in different types of neurodegeneration. CoQ10 is detectable in retina and declines with ageing. Early studies showed treatment of CoQ10 improved visual function in patients with age-related macular degeneration. In glaucomatous models, CoQ10 exposure protected ganglion cell death from environmental stress; in glaucoma patients, CoQ10 treatment demonstrated beneficial effects on function of inner retina and enhancement of visual cortical response. Since oxidative stress also plays a critical role in the pathogenesis of diabetic retinopathy and retinitis pigmentosa, CoQ10 is a therapeutic target for both conditions.. A wide range of evidence supports a role of CoQ10 in retinal diseases through inhibiting production of reactive oxygen species and protecting neuroretinal cells from oxidative damage.

Topics: Animals; Humans; Retinal Diseases; Ubiquinone

The evaluation of the short-term effect of topically applied coenzyme Q10 (CoQ10) on retina and choroid in Alzheimer's disease (AD) was aimed in this study.. Randomized controlled study included a total of 93 patients, 62 of whom with AD. Thirty (32.3%) AD patients received treatment (Group 1), 32 (34.4%) AD patients observed without treatment (Group 2), and Group 3 included 31 (33.3%) healthy controls (HC). Neurological and ophthalmological examinations including optical coherence tomography (OCT) were executed.. Retinal nerve fiber layer (RNFL) thickness in all quadrants increased following CoQ10 treatment in Group 1; however significant rise yielded in average and temporal quadrant RNFL thickness. Average and superonasal sector ganglion cell-inner plexiform layer (GCIPL) thickness increased significantly following CoQ10 treatment. The correlation analysis between difference in pre- and posttreatment OCT values in Group 1 revealed that rise in average RNFL thickness was inversely correlated with duration of the disease and rise in average GCIPL thickness and superonasal sector thickness was inversely correlated with severity of the disease.. Short-term topical CoQ10 resulted in improvement in AD related retinal ganglion cell (RGC) loss which may reflect the salvage of some RGCs in the reversible transitional phase. More bioavailability through intravitreal route of administration and longer duration of effect with sustained release forms may possibly help enhalting the RGC loss, especially incipience of neurodegenerative diseases.

Topics: Aged; Alzheimer Disease; Choroid; Dose-Response Relationship, Drug; Female; Humans; Male; Ophthalmic Solutions; Retinal Diseases; Retinal Ganglion Cells; Salvage Therapy; Tomography, Optical Coherence; Ubiquinone; Vitamins

Topics: Brain Diseases; Humans; Mutation; Renal Insufficiency; Retinal Diseases; Ubiquinone

In this study, it was aimed to investigate the effect of coenzyme Q10 (CoQ10) on cisplatin-induced oxidative retinal damage in rats biochemically and histopathologically.. Thirty male Wistar albino rats were divided into 3 groups randomly: untreated control (C group), only 2.5 mg/kg cisplatin daily administrated group for 2 weeks (CP group), 2.5 mg/kg cisplatin + 20 mg/kg orally CoQ10 daily administrated group for 2 weeks (CoQC group). At the end of experimental period, blood samples obtained before sacrification for the biochemical examination of serum malondialdehyde (MDA), total glutathione (tGSH), total oxidant system (TOS), total antioxidant systemic (TAS) levels and after eyes were removed for examined histopathology.. As a result of our study, severe histopathological damage was detected in the retinal tissue of the cisplatin group with serum malondialdehyde (MDA) and total oxidant system (TOS) levels were high and total glutathione (tGSH) and total antioxidant systemic (TAS) levels were low. However, it was observed that the histopathological damage associated with cisplatin was decreased in the retinal tissue of the CoQ10 group, which inhibited the increase in blood serum MDA/TOS levels and decrease in tGSH/TAS levels.. The biochemical and histopathological results of our study were compatible with each other, so we concluded that the damage to the rat retinal tissue caused by cisplatin may be reversible with coenzyme.

Topics: Administration, Oral; Animals; Antioxidants; Cisplatin; Disease Models, Animal; Humans; Male; Oxidative Stress; Rats; Retina; Retinal Diseases; Ubiquinone

The retinal pathophysiology of methanol intoxication is that formate inhibits retinal mitochondrial function and increases oxidative stress.. To investigate the effect of coenzyme Q10 and curcumin on chronic methanol intoxication causing retinopathy in rats.. The authors designed an experimental study of chronic methanol intoxication in rats depleted of folate with methotrexate. The studied group received methanol (2 mg/kg body weight in saline by intraperitoneal injection) and methotrexate (0.1 mg/kg body weight in saline by subcutaneous injection) every other day for ten weeks to induce chronic methanol intoxication, while another group received saline as vehicle and served as control group. The studied rats were confirmed to develop significant retinopathy after 10 weeks and then assigned to three treatment arms: either corn oil (as control) or coenzyme Q10 (20 mg/kg/day) or Curcuma longa extract (2.5 mg/kg/day) for four weeks. Eyes were enucleated and the retinal tissue was prepared for histological examination. The sections were evaluated by an experienced pathologist and blinded to the experimental conditions.. Histological analysis revealed that animals treated with both methanol and methotrexate showed vacuolation of photoreceptor inner segment and disaggregation of cells in the inner and outer nuclear layers of the retina compared to a normal histological appearance in control animals. The retinal histology in the experimental animals with administration of Coenzyme Q10 or Curcuma longa extract appeared essentially normal and this was not found in the experimental animals which received corn oil.. Coenzyme Q10 and curcumin administration improves retinal histology by reversing the pathological changes due to chronic methanol and establish a morphologically normal retina.

Topics: Animals; Anti-Inflammatory Agents, Non-Steroidal; Curcumin; Male; Methanol; Rats; Rats, Sprague-Dawley; Retinal Diseases; Solvents; Ubiquinone; Vitamins

To evaluate if coenzyme Q10 (CoQ10) can protect retinal ganglion cells (RGCs) from apoptosis and, when instilled as eye drops on the cornea, if it can reach the retina and exert its antiapoptotic activity in this area in a mouse model of kainate (KA)-induced retinal damage.. Rat primary or cultured RGCs were subjected to glutamate (50 μM) or chemical hypoxia (Antimycin A, 200 μM) or serum withdrawal (FBS, 0.5%) in the presence or absence of CoQ10 (10 μM). Cell viability was evaluated by light microscopy and fluorescence-activated cell sorting analyses. Apoptosis was evaluated by caspase 3/7 activity and mitochondrion depolarization tetramethylrhodamine ethyl ester analysis. CoQ10 transfer to the retina following its instillation as eye drops on the cornea was quantified by HPLC. Retinal protection by CoQ10 (10 μM) eye drops instilled on the cornea was then evaluated in a mouse model of KA-induced excitotoxic retinal cell apoptosis by cleaved caspase 3 immunohistofluorescence, caspase 3/7 activity assays, and quantification of inhibition of RGC loss.. CoQ10 significantly increased viable cells by preventing RGC apoptosis. Furthermore, when topically applied as eye drops to the cornea, it reached the retina, thus substantially increasing local CoQ10 concentration and protecting retinal layers from apoptosis.. The ability of CoQ10 eye drops to protect retinal cells from apoptosis in the mouse model of KA-induced retinal damage suggests that topical CoQ10 may be evaluated in designing therapies for treating apoptosis-driven retinopathies.

Topics: Administration, Topical; Animals; Antimycin A; Apoptosis; Caspase 3; Caspase 7; Cell Count; Cell Survival; Cells, Cultured; Chromatography, High Pressure Liquid; Cornea; Disease Models, Animal; Dose-Response Relationship, Drug; Fluorescent Antibody Technique, Indirect; Glutamic Acid; Kainic Acid; Male; Mice; Mice, Inbred C57BL; Mitochondria; Ophthalmic Solutions; Rabbits; Rats; Rats, Wistar; Retina; Retinal Diseases; Retinal Ganglion Cells; Time Factors; Ubiquinone; Vitamins

Recent studies support a role for excitotoxicity in the development of retinal ganglion cell (RGC) damage in subjects suffering from glaucoma. Coenzyme Q10 (CoQ10), an essential cofactor of the electron transport chain, has been reported to afford neuroprotection, preventing the formation of the mitochondrial permeability transition pore. Using an established animal model of retinal ischemia/reperfusion here, we show that synaptic glutamate increases at 130min from beginning of reperfusion and delayed apoptosis in the RGC layer is seen at 24h. Intraocular administration of CoQ10 minimizes glutamate increase and affords neuroprotection, suggesting that oxidative stress and energy failure might be implicated in the mechanisms of RGC death.

Topics: Administration, Topical; Animals; Coenzymes; Glutamic Acid; In Situ Nick-End Labeling; Intraocular Pressure; Ischemia; Male; Microdialysis; Rats; Rats, Wistar; Retinal Diseases; Retinal Ganglion Cells; Ubiquinone

We report severe coenzyme Q10 deficiency of muscle in a 4-year-old boy presenting with progressive muscle weakness, seizures, cerebellar syndrome, and a raised cerebro-spinal fluid lactate concentration. State-3 respiratory rates of muscle mitochondria with glutamate, pyruvate, palmitoylcarnitine, and succinate as respiratory substrates were markedly reduced, whereas ascorbate/N,N,N',N'-tetramethyl-p-phenylenediamine were oxidized normally. The activities of complexes I, II, III and IV of the electron transport chain were normal, but the activities of complexes I+III and II+III, both systems requiring coenzyme Q10 as an electron carrier, were dramatically decreased. These results suggested a defect in the mitochondrial coenzyme Q10 content. This was confirmed by the direct assessment of coenzyme Q10 level by high-performance liquid chromatography in patient's muscle homogenate and isolated mitochondria, revealing levels of 16% and 6% of the control values, respectively. We did not find any impairment of the respiratory chain either in a lymphoblastoid cell line or in skin cultured fibroblasts from the patient, suggesting that the coenzyme Q10 depletion was tissue-specific. This is a new case of a muscle deficiency of mitochondrial coenzyme Q in a patient suffering from an encephalomyopathy.

Topics: Cerebellar Ataxia; Child, Preschool; Coenzymes; Electron Transport; Epilepsy; Humans; Kinetics; Lactic Acid; Male; Mitochondria, Muscle; Mitochondrial Encephalomyopathies; Muscle, Skeletal; Polarography; Retinal Diseases; Ubiquinone