{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T01:59:18Z","timestamp":1788919158881,"version":"build-2803163510"},"reference-count":91,"publisher":"MDPI AG","issue":"23","license":[{"start":{"date-parts":[[2020,12,6]],"date-time":"2020-12-06T00:00:00Z","timestamp":1607212800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Coenzyme Q10 (CoQ10) is a ubiquitous cofactor in the body, operating in the inner mitochondrial membrane, where it plays a vital role in the generation of adenosine triphosphate (ATP) through the electron transport chain (ETC). In addition to this, CoQ10 serves as an antioxidant, protecting the cell from oxidative stress by reactive oxygen species (ROS) as well as maintaining a proton (H+) gradient across lysosome membranes to facilitate the breakdown of cellular waste products. Through the process of ageing, the body becomes deficient in CoQ10, resulting in several systemic manifestations. On a cellular level, one of the consequences of CoQ10 deficiency is apoptosis, which can be visualised in tissues of the central nervous system (CNS). Diseases affecting the retina and brain such as age-related macular degeneration (AMD), glaucoma, Alzheimer\u2019s disease (AD) and Parkinson\u2019s disease (PD) have shown defects in cellular biochemical reactions attributed to reduced levels of CoQ10. Through further research into the pathogenesis of such conditions, the effects of CoQ10 deficiency can be counteracted through supplementation, early detection and intervention.<\/jats:p>","DOI":"10.3390\/ijms21239299","type":"journal-article","created":{"date-parts":[[2020,12,7]],"date-time":"2020-12-07T12:24:39Z","timestamp":1607343879000},"page":"9299","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":74,"title":["Cellular Consequences of Coenzyme Q10 Deficiency in Neurodegeneration of the Retina and Brain"],"prefix":"10.3390","volume":"21","author":[{"given":"Haider","family":"Manzar","sequence":"first","affiliation":[{"name":"Imperial College Ophthalmology Research Group, Western Eye Hospital, 153-173 Marylebone Road, Marylebone, London NW1 5QH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dalia","family":"Abdulhussein","sequence":"additional","affiliation":[{"name":"Imperial College Ophthalmology Research Group, Western Eye Hospital, 153-173 Marylebone Road, Marylebone, London NW1 5QH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy E.","family":"Yap","sequence":"additional","affiliation":[{"name":"Imperial College Ophthalmology Research Group, Western Eye Hospital, 153-173 Marylebone Road, Marylebone, London NW1 5QH, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. Francesca","family":"Cordeiro","sequence":"additional","affiliation":[{"name":"Imperial College Ophthalmology Research Group, Western Eye Hospital, 153-173 Marylebone Road, Marylebone, London NW1 5QH, UK"},{"name":"Glaucoma &amp; Retinal Neurodegeneration Research Group, Institute of Ophthalmology, University College London, London EC1V 9EL, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2020,12,6]]},"reference":[{"key":"ref_1","unstructured":"Silverman, H.M., Romano, J., and Elmer, G. (1993). The Vitamin Book, Bantam Books."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1016\/j.bbabio.2016.03.036","article-title":"Coenzyme Q biosynthesis in health and disease","volume":"1857","author":"Acosta","year":"2016","journal-title":"Biochimica Biophysica Acta (BBA)-Bioenerg."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Nohl, H., and Gille, L. (2001). The role of coenzyme Q in lysosomes. Coenzyme Q: Molecular Mechanisms in Health and Disease, CRC Press.","DOI":"10.1201\/9781420036701.ch6"},{"key":"ref_4","unstructured":"Challem, J. (The Nutritional Reporter, 1996). Coenzyme Q10: It may just be the miracle vitamin of the 1990s, The Nutritional Reporter."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"9216","DOI":"10.3390\/ijms12129216","article-title":"Hormonal influence on coenzyme Q10 levels in blood plasma","volume":"12","author":"Mancini","year":"2011","journal-title":"Int. J. Mol. Sci."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1172\/JCI31423","article-title":"Mutations in coenzyme Q 10 biosynthetic genes","volume":"117","author":"Quinzii","year":"2007","journal-title":"J. Clin. Investig."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"361","DOI":"10.1002\/biof.155","article-title":"Primary and secondary CoQ10 deficiencies in humans","volume":"37","author":"Quinzii","year":"2011","journal-title":"Biofactors"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1111\/nure.12011","article-title":"Primary and secondary coenzyme Q10 deficiency: The role of therapeutic supplementation","volume":"71","author":"Potgieter","year":"2013","journal-title":"Nutr. Rev."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1016\/0003-9861(92)90511-T","article-title":"Distribution and redox state of ubiquinones in rat and human tissues","volume":"295","author":"Appelkvist","year":"1992","journal-title":"Arch. Biochem. Biophys."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1016\/S0009-8981(03)00137-2","article-title":"Plasma coenzyme Q10 reference intervals, but not redox status, are affected by gender and race in self-reported healthy adults","volume":"332","author":"Miles","year":"2003","journal-title":"Clin. Chim. Acta"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"3733","DOI":"10.1096\/fj.09-152728","article-title":"Reactive oxygen species, oxidative stress, and cell death correlate with level of CoQ10 deficiency","volume":"24","author":"Quinzii","year":"2010","journal-title":"FASEB J."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"525","DOI":"10.3389\/fphys.2017.00525","article-title":"The role of sulfide oxidation impairment in the pathogenesis of primary CoQ deficiency","volume":"8","author":"Quinzii","year":"2017","journal-title":"Front. Physiol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/S0022-2275(20)37636-7","article-title":"Measurement of the ratio between the reduced and oxidized forms of coenzyme Q10 in human plasma as a possible marker of oxidative stress","volume":"37","author":"Lagendijk","year":"1996","journal-title":"J. Lipid Res."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"K\u00fchlbrandt, W. (2015). Structure and function of mitochondrial membrane protein complexes. BMC Biol., 13.","DOI":"10.1186\/s12915-015-0201-x"},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"Heaton, R.A., Heales, S., Rahman, K., Sexton, D.W., and Hargreaves, I. (2020). The Effect of Cellular Coenzyme Q10 Deficiency on Lysosomal Acidification. J. Clin. Med., 9.","DOI":"10.3390\/jcm9061923"},{"key":"ref_16","doi-asserted-by":"crossref","unstructured":"De Barcelos, I.P.D., and Haas, R.H. (2019). Coq10 and ageing. Biology, 8.","DOI":"10.3390\/biology8020028"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1067\/mpd.2001.117575","article-title":"Neonatal presentation of coenzyme Q10 deficiency","volume":"139","author":"Rahman","year":"2001","journal-title":"J. Pediatrics"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"2037","DOI":"10.1093\/brain\/awm054","article-title":"The myopathic form of coenzyme Q10 deficiency is caused by mutations in the electron-transferring-flavoprotein dehydrogenase (ETFDH) gene","volume":"130","author":"Gempel","year":"2007","journal-title":"Brain"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1016\/S0140-6736(00)02531-9","article-title":"Quinone-responsive multiple respiratory-chain dysfunction due to widespread coenzyme Q10 deficiency","volume":"356","author":"Appelkvist","year":"2000","journal-title":"Lancet"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1172\/JCI29089","article-title":"Prenyldiphosphate synthase, subunit 1 (PDSS1) and OH-benzoate polyprenyltransferase (COQ2) mutations in ubiquinone deficiency and oxidative phosphorylation disorders","volume":"117","author":"Mollet","year":"2007","journal-title":"J. Clin. Investig."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"4256","DOI":"10.1093\/hmg\/ddw257","article-title":"The COQ2 genotype predicts the severity of coenzyme Q10 deficiency","volume":"25","author":"Desbats","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1814","DOI":"10.1167\/iovs.08-2656","article-title":"Coenzyme Q10 in the human retina","volume":"50","author":"Qu","year":"2009","journal-title":"Investig. Ophthalmol. Vis. Sci."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1111\/j.1471-4159.1990.tb01889.x","article-title":"Lipid compositions of different regions of the human brain during aging","volume":"54","author":"Edlund","year":"1990","journal-title":"J. Neurochem."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1139\/o92-065","article-title":"Ubiquinone, dolichol, and cholesterol metabolism in aging and Alzheimer\u2019s disease","volume":"70","author":"Edlund","year":"1992","journal-title":"Biochem. Cell Biol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"17","DOI":"10.3164\/jcbn.17.124","article-title":"Increased oxidative stress and coenzyme Q10 deficiency in centenarians","volume":"63","author":"Nagase","year":"2018","journal-title":"J. Clin. Biochem. Nutr."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1392","DOI":"10.1136\/bmj.305.6866.1392","article-title":"Serum antioxidant vitamins and risk of cataract","volume":"305","author":"Knekt","year":"1992","journal-title":"BMJ"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"93","DOI":"10.2147\/vhrm.1.2.93.64080","article-title":"Are we over oxidized? Oxidative stress, cardiovascular disease, and the future of intervention studies with antioxidants","volume":"1","author":"Dusting","year":"2005","journal-title":"Vasc. Health Risk Manag."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"411","DOI":"10.2174\/187152707783399201","article-title":"Neuronal Death and Survival Under Oxidative Stress in Alzheimer and Parkinson Diseases","volume":"6","author":"Nunomura","year":"2008","journal-title":"CNS Neurol. Disord. Drug Targets"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/S0005-2728(98)00120-0","article-title":"Role of mitochondria in oxidative stress and ageing","volume":"1366","author":"Lenaz","year":"1998","journal-title":"Biochim. Biophys. Acta Bioenerg."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1159\/000050826","article-title":"Does coenzyme Q10 play a role in opposing oxidative stress in patients with age-related macular degeneration?","volume":"215","author":"Blasi","year":"2001","journal-title":"Ophthalmologica"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1001\/jamaneurol.2014.131","article-title":"A randomized clinical trial of high-dosage coenzyme Q10 in early Parkinson disease: No evidence of benefit","volume":"71","author":"Beal","year":"2014","journal-title":"JAMA Neurol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1016\/S0074-7742(07)82022-8","article-title":"Retinal damage caused by high intraocular pressure\u2013induced transient ischemia is prevented by coenzyme Q10 in rat","volume":"82","author":"Nucci","year":"2007","journal-title":"Int. Rev. Neurobiol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1016\/S0079-6123(08)01139-4","article-title":"Rational basis for the development of coenzyme Q10 as a neurotherapeutic agent for retinal protection","volume":"173","author":"Russo","year":"2008","journal-title":"Prog. Brain Res."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1167\/iovs.13-12564","article-title":"Coenzyme Q10 inhibits glutamate excitotoxicity and oxidative stress\u2013mediated mitochondrial alteration in a mouse model of glaucoma","volume":"55","author":"Lee","year":"2014","journal-title":"Investig. Ophthalmol. Vis. Sci."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1080\/10715760600617843","article-title":"Coenzyme Q10: Absorption, tissue uptake, metabolism and pharmacokinetics","volume":"40","author":"Bhagavan","year":"2006","journal-title":"Free Radic. Res."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1016\/j.ejphar.2016.04.030","article-title":"New strategies for neuroprotection in glaucoma, a disease that affects the central nervous system","volume":"787","author":"Nucci","year":"2016","journal-title":"Eur. J. Pharmacol."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"3754","DOI":"10.2174\/0929867325666180307114332","article-title":"Neurodegenerative process linking the eye and the brain","volume":"26","author":"Mancino","year":"2019","journal-title":"Curr. Med. Chem."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"8295","DOI":"10.1167\/iovs.12-10374","article-title":"Coenzyme Q10 instilled as eye drops on the cornea reaches the retina and protects retinal layers from apoptosis in a mouse model of kainate-induced retinal damage","volume":"53","author":"Lulli","year":"2012","journal-title":"Investig. Ophthalmol. Vis. Sci."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1016\/j.mito.2017.05.010","article-title":"Topical Coenzyme Q10 demonstrates mitochondrial-mediated neuroprotection in a rodent model of ocular hypertension","volume":"36","author":"Davis","year":"2017","journal-title":"Mitochondrion"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1177\/1120672118779486","article-title":"Evaluation of oxidative stress in pseudo-exfoliative glaucoma patients treated with and without topical coenzyme Q10 and vitamin E","volume":"29","author":"Ozates","year":"2019","journal-title":"Eur. J. Ophthalmol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"2506","DOI":"10.1007\/s12325-019-01023-3","article-title":"Evaluating the effects of an ophthalmic solution of coenzyme Q10 and vitamin E in open-angle glaucoma patients: A study protocol","volume":"36","author":"Quaranta","year":"2019","journal-title":"Adv. Ther."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1016\/j.atherosclerosis.2014.09.017","article-title":"Serum coenzyme Q10 and risk of disabling dementia: The Circulatory Risk in Communities Study (CIRCS)","volume":"237","author":"Yamagishi","year":"2014","journal-title":"Atherosclerosis"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1016\/j.neuro.2011.12.005","article-title":"Coenzyme Q10 protects against amyloid beta-induced neuronal cell death by inhibiting oxidative stress and activating the P13K pathway","volume":"33","author":"Choi","year":"2012","journal-title":"Neurotoxicology"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1007\/s12031-007-9033-7","article-title":"Coenzyme Q10 attenuates \u03b2-amyloid pathology in the aged transgenic mice with Alzheimer presenilin 1 mutation","volume":"34","author":"Yang","year":"2008","journal-title":"J. Mol. Neurosci."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"211","DOI":"10.3233\/JAD-2011-110209","article-title":"Coenzyme Q10 decreases amyloid pathology and improves behavior in a transgenic mouse model of Alzheimer\u2019s disease","volume":"27","author":"Dumont","year":"2011","journal-title":"J. Alzheimer\u2019s Dis."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1016\/j.neurobiolaging.2004.08.011","article-title":"Oxidative state in platelets and erythrocytes in aging and Alzheimer\u2019s disease","volume":"26","author":"Kawamoto","year":"2005","journal-title":"Neurobiol. Aging"},{"key":"ref_47","first-page":"882","article-title":"Coenzyme Q10 and nicotinamide block striatal lesions produced by the mitochondrial toxin malonate","volume":"36","author":"Beal","year":"1994","journal-title":"Ann. Neurol. Off. J. Am. Neurol. Assoc. Child Neurol. Soc."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"836","DOI":"10.1001\/archneurol.2012.85","article-title":"Antioxidants for Alzheimer disease: A randomized clinical trial with cerebrospinal fluid biomarker measures","volume":"69","author":"Galasko","year":"2012","journal-title":"Arch. Neurol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1016\/0167-4943(92)90060-H","article-title":"Idebenone in senile dementia of Alzheimer type: A multicentre study","volume":"15","author":"Senin","year":"1992","journal-title":"Arch. Gerontol. Geriatr."},{"key":"ref_50","first-page":"161","article-title":"Idebenone, a new drug in the treatment of cognitive impairment in patients with dementia of the Alzheimer type","volume":"9","author":"Bergamasco","year":"1994","journal-title":"Funct. Neurol."},{"key":"ref_51","first-page":"S125","article-title":"Criteria for diagnosing Parkinson\u2019s disease","volume":"32","author":"Calne","year":"1992","journal-title":"Ann. Neurol. Off. J. Am. Neurol. Assoc. Child Neurol. Soc."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"II3","DOI":"10.1007\/PL00007758","article-title":"Pathoanatomy of Parkinson\u2019s disease","volume":"247","author":"Braak","year":"2000","journal-title":"J. Neurol."},{"key":"ref_53","doi-asserted-by":"crossref","unstructured":"Forno, L.S. (1988). The Neuropathology Of Parkinson\u2019S Disease. Progress in Parkinson Research, Springer.","DOI":"10.1007\/978-1-4613-0759-4_2"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1046\/j.1469-1809.2001.6520111.x","article-title":"Genetics of Parkinsonism: A review","volume":"65","author":"Vaughan","year":"2001","journal-title":"Ann. Hum. Genet."},{"key":"ref_55","unstructured":"Sherer, T. (2020, November 06). Pathogenesis of Parkinson\u2019s Disease. Available online: https:\/\/europepmc.org\/article\/med\/11569943."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1146\/annurev.neuro.22.1.123","article-title":"Etiology and pathogenesis of Parkinson\u2019s disease","volume":"22","author":"Olanow","year":"1999","journal-title":"Annu. Rev. Neurosci."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/S1474-4422(07)70327-7","article-title":"Mitochondria in the aetiology and pathogenesis of Parkinson\u2019s disease","volume":"7","author":"Schapira","year":"2008","journal-title":"Lancet Neurol."},{"key":"ref_58","first-page":"57","article-title":"Oxidative stress in Parkinson\u2019s disease and other neurodegenerative disorders","volume":"44","author":"Jenner","year":"1996","journal-title":"Pathol. Biol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1002\/ana.410360305","article-title":"Alterations in glutathione levels in Parkinson\u2019s disease and other neurodegenerative disorders affecting basal ganglia","volume":"36","author":"Sian","year":"1994","journal-title":"Ann. Neurol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1002\/ana.410260606","article-title":"Abnormalities of the electron transport chain in idiopathic parkinson\u2019s disease","volume":"26","author":"Parker","year":"1989","journal-title":"Ann. Neurol."},{"key":"ref_61","doi-asserted-by":"crossref","unstructured":"Turner, C., and Schapira, A.H.V. (2001). Mitochondrial dysfunction in neurodegenerative disorders and ageing. Advances in Experimental Medicine and Biology, Springer.","DOI":"10.1007\/978-1-4615-1249-3_19"},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1002\/ana.410320612","article-title":"Platelet mitochondria function in Parkinson\u2019s disease","volume":"32","author":"Krige","year":"1992","journal-title":"Ann. Neurol."},{"key":"ref_63","first-page":"35","article-title":"Mechanisms of MPTP toxicity","volume":"13","author":"Przedborski","year":"1998","journal-title":"Mov. Disord."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1126\/science.6823561","article-title":"Chronic parkinsonism in humans due to a product of meperidine-analog synthesis","volume":"219","author":"Ballard","year":"1983","journal-title":"Science"},{"key":"ref_65","doi-asserted-by":"crossref","unstructured":"Fahn, S. (2006). Levodopa in the treatment of Parkinson\u2019s disease. Oxidative Stress and Neuroprotection, Springer.","DOI":"10.1007\/978-3-211-33328-0_1"},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1212\/WNL.45.2.344","article-title":"Carbidopa\/levodopa and selegiline do not affect platelet mitochondrial function in early parkinsonism","volume":"45","author":"Shults","year":"1995","journal-title":"Neurology"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1002\/ana.410420221","article-title":"Coenzyme Q10 levels correlate with the activities of complexes I and II\/III in mitochondria from Parkinsonian and nonparkinsonian subjects","volume":"42","author":"Shults","year":"1997","journal-title":"Ann. Neurol."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1016\/S0006-8993(97)01192-X","article-title":"Coenzyme Q10 attenuates the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) induced loss of striatal dopamine and dopaminergic axons in aged mice","volume":"783","author":"Beal","year":"1998","journal-title":"Brain Res."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"793","DOI":"10.1212\/WNL.50.3.793","article-title":"Absorption, tolerability, and effects on mitochondrial activity of oral coenzyme Q10 in parkinsonian patients","volume":"50","author":"Shults","year":"1998","journal-title":"Neurology"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"1541","DOI":"10.1001\/archneur.59.10.1541","article-title":"Effects of coenzyme Q 10 in early Parkinson disease: Evidence of slowing of the functional decline","volume":"59","author":"Shults","year":"2002","journal-title":"Arch Neurol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1016\/j.ijpharm.2005.09.040","article-title":"Therapeutic potential of controlled drug delivery systems in neurodegenerative diseases","volume":"314","author":"Popovic","year":"2006","journal-title":"Int. J. Pharm."},{"key":"ref_72","first-page":"1","article-title":"Intrastriatal administration of coenzyme Q10 enhances neuroprotection in a Parkinson\u2019s disease rat model","volume":"10","author":"Park","year":"2020","journal-title":"Sci. Rep."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1002\/mds.870110317","article-title":"Assessment of coenzyme Q10 tolerability in Huntington\u2019s disease","volume":"11","author":"Feigin","year":"1996","journal-title":"Mov. Disord. Off. J. Mov. Disord. Soc."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1592","DOI":"10.1523\/JNEUROSCI.22-05-01592.2002","article-title":"Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington\u2019s disease","volume":"22","author":"Ferrante","year":"2002","journal-title":"J. Neurosci."},{"key":"ref_75","first-page":"160","article-title":"Energy metabolism defects in Huntington\u2019s disease and effects of coenzyme Q10","volume":"41","author":"Koroshetz","year":"1997","journal-title":"Ann. Neurol. Off. J. Am. Neurol. Assoc. Child Neurol. Soc."},{"key":"ref_76","doi-asserted-by":"crossref","unstructured":"Huntington Study Group (2001). A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington\u2019s disease. Neurology, 57, 397\u2013404.","DOI":"10.1212\/WNL.57.3.397"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1016\/0022-510X(90)90015-F","article-title":"Ubidecarenone in the treatment of mitochondrial myopathies: A multi-center double-blind trial","volume":"100","author":"Bresolin","year":"1990","journal-title":"J. Neurol. Sci."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1093\/brain\/104.3.589","article-title":"Friedreich\u2019s ataxia: A clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features","volume":"104","author":"Harding","year":"1981","journal-title":"Brain"},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"1169","DOI":"10.1056\/NEJM199610173351601","article-title":"Clinical and Genetic Abnormalities in Patients with Friedreich\u2019s Ataxia","volume":"335","author":"Cossee","year":"1996","journal-title":"N. Engl. J. Med."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1186\/s40673-017-0062-x","article-title":"Friedreich Ataxia: Current status and future prospects","volume":"4","year":"2017","journal-title":"Cerebellum Ataxias"},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"1423","DOI":"10.1126\/science.271.5254.1423","article-title":"Friedreich\u2019s ataxia: Autosomal recessive disease caused by an intronic GAA triplet repeat expansion","volume":"271","author":"Campuzano","year":"1996","journal-title":"Science"},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"1771","DOI":"10.1093\/hmg\/6.11.1771","article-title":"Frataxin is reduced in Friedreich ataxia patients and is associated with mitochondrial membranes","volume":"6","author":"Campuzano","year":"1997","journal-title":"Hum. Mol. Genet."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1093\/hmg\/8.3.425","article-title":"The Friedreich\u2019s ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis","volume":"8","author":"Wong","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1093\/hmg\/9.2.275","article-title":"Clinical, biochemical and molecular genetic correlations in Friedreich\u2019s ataxia","volume":"9","author":"Bradley","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1038\/ng0897-345","article-title":"Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin","volume":"16","author":"Koutnikova","year":"1997","journal-title":"Nat. Genet."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"1709","DOI":"10.1126\/science.276.5319.1709","article-title":"Regulation of mitochondrial iron accumulation by Yfh1p, a putative homolog of frataxin","volume":"276","author":"Babcock","year":"1997","journal-title":"Science"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1016\/S0014-5793(97)00734-5","article-title":"Deletion of the yeast homologue of the human gene associated with Friedreich\u2019s ataxia elicits iron accumulation in mitochondria","volume":"411","author":"Foury","year":"1997","journal-title":"FEBS Lett."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"580","DOI":"10.1016\/j.ygeno.2006.06.015","article-title":"GAA repeat expansion mutation mouse models of Friedreich ataxia exhibit oxidative stress leading to progressive neuronal and cardiac pathology","volume":"88","author":"Pinto","year":"2006","journal-title":"Genomics"},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1002\/ana.1001","article-title":"Antioxidant treatment improves in vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich\u2019s ataxia","volume":"49","author":"Lodi","year":"2001","journal-title":"Ann. Neurol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1001\/archneur.62.4.621","article-title":"Antioxidant treatment of patients with Friedreich ataxia: Four-year follow-up","volume":"62","author":"Hart","year":"2005","journal-title":"Arch Neurol."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"1371","DOI":"10.1111\/j.1468-1331.2008.02318.x","article-title":"Coenzyme Q10 and vitamin e deficiency in Friedreich\u2019s ataxia: Predictor of efficacy of vitamin e and coenzyme Q10 therapy","volume":"15","author":"Cooper","year":"2008","journal-title":"Eur. J. Neurol."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/21\/23\/9299\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T10:41:34Z","timestamp":1760179294000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/21\/23\/9299"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,12,6]]},"references-count":91,"journal-issue":{"issue":"23","published-online":{"date-parts":[[2020,12]]}},"alternative-id":["ijms21239299"],"URL":"https:\/\/doi.org\/10.3390\/ijms21239299","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,12,6]]}}}