ArticleJournal of neuropathology and experimental neurology2016
Decreased Coenzyme Q10 Levels in Multiple System Atrophy Cerebellum.
Open access · bronzeAbstract read
In one paragraph
Article in Journal of neuropathology and experimental neurology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 46 papers.
0numbers the graph read from it
0cells of the map it votes in
46citing papers in PubMed
4.2field-weighted citation impact, top 6% of its field
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
46 citing papers in PubMed, 69 citations in OpenAlex.
- Regional reduction of high-energy phosphorus metabolites characterizes the cerebellar subtype of multiple system atrophy.NPJ Parkinson's disease · 2026Article
- Drug repurposing for disease-modifying effects in multiple system atrophy.Translational neurodegeneration · 2026Review
- Spatial mapping of CoQ10 repletion by BPM31510 in a genetic mouse model (Coq4Journal of lipid research · 2026Article
- Proximity proteomics reveals unique and shared pathological features between multiple system atrophy and Parkinson's disease.Acta neuropathologica communications · 2025Article
- Coenzyme QFree radical biology & medicine · 2025Article
- Genetic profiles of multiple system atrophy revealed by exome sequencing, long-read sequencing and spinocerebellar ataxia repeat expansion analysis.European journal of neurology · 2024Article
- Understanding coenzyme Q.Physiological reviews · 2024Review
- Comparing alpha-synuclein-interactomes between multiple systems atrophy and Parkinson's disease reveals unique and shared pathological features.bioRxiv : the preprint server for biology · 2024Article
- Probing the diagnostic values of plasma cf-nDNA and cf-mtDNA for Parkinson's disease and multiple system atrophy.Frontiers in neuroscience · 2024Article
- Multidimensional biomarkers for multiple system atrophy: an update and future directions.Translational neurodegeneration · 2023Review
- High-dose ubiquinol supplementation in multiple-system atrophy: a multicentre, randomised, double-blinded, placebo-controlled phase 2 trial.EClinicalMedicine · 2023Article
- Parkinson's Disease, Parkinsonisms, and Mitochondria: the Role of Nuclear and Mitochondrial DNA.Current neurology and neuroscience reports · 2023Review
- Genetics of Multiple System Atrophy and Progressive Supranuclear Palsy: A Systemized Review of the Literature.International journal of molecular sciences · 2023Review
- Glutathione Depletion and MicroRNA Dysregulation in Multiple System Atrophy: A Review.International journal of molecular sciences · 2022Review
- Coenzyme Q10: Role in Less Common Age-Related Disorders.Antioxidants (Basel, Switzerland) · 2022Review
- Mitochondrial Dysfunction and Neurodegenerative Disorders: Role of Nutritional Supplementation.International journal of molecular sciences · 2022Review
- Multiple system atrophy.Nature reviews. Disease primers · 2022Review
- Coenzyme Q10 and Parkinsonian Syndromes: A Systematic Review.Journal of personalized medicine · 2022Review
- Mechanisms and Therapeutic Effects of Benzoquinone Ring Analogs in Primary CoQ Deficiencies.Antioxidants (Basel, Switzerland) · 2022Review
- Coenzyme Q10, Ageing and the Nervous System: An Overview.Antioxidants (Basel, Switzerland) · 2021Review
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
14 authors at 6 institutions in 2 countries.
Emanuele BarcaFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Giulio KleinerFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Guomei TangFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Marcello ZiosiFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Saba TadesseFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Eliezer MasliahFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Elan D LouisFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Phyllis FaustFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Un J KangFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Jose TorresFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Etty P CortesFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Jean-Paul G VonsattelFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Sheng-Han KuoFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV).
Catarina M QuinziiFrom the Department of Neurology, College of Physicians and Surgeons, Columbia University, New York (EB, GK, GT, MZ, ST, UJK, S-HK, CMQ); UOC of Neurology and Neuromuscular Disorders, Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy (EB); Department of Neuroscience and Pathology, University of California, San Diego, California (EM); Department of Neurology, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, Connecticut (EDL); Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, Connecticut (EDL); Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York (PF, JT, EPC, J-PGV); and Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Department of Pathology and Cell Biology, College of Physicians and Surgeons, Columbia University, New York (JT, EPC, J-PGV). sk3295@columbia.edu.
Columbia University · USUniversity of Messina · ITYale University · USColumbia University Irving Medical Center · USNewYork–Presbyterian Hospital · USUniversity of California, San Diego · US
Funding
Yale Clinical and Translational Science Award (U Component)UL1TR001863 · NCATS · YALE UNIVERSITY · PI John H. Krystal, LUCILA OHNO-MACHADO · 2016 to 2026
$102.9MTrue Metal fac coreP30ES009089 · NIEHS · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI MATTHEW S PERZANOWSKI · 1998 to 2026
$44.7MClinical Pathological Study of Cognitive Impairment in Essential TremorR01NS086736 · NINDS · YALE UNIVERSITY · PI COSENTINO, STEPHANIE ANN, LOUIS, ELAN D · 2014 to 2023
$8.1MTech-CoreP01HD080642 · NICHD · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI QUINZII, CATARINA M. · 2014 to 2018
$7.8MENVIRONMENTAL EPIDEMIOLOGY OF ESSENTIAL TREMORR01NS039422 · NINDS · YALE UNIVERSITY · PI LOUIS, ELAN D · 2000 to 2013
$7.7MIdentification of Susceptibility Genes for Essential TremorR01NS073872 · NINDS · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI CLARK, LORRAINE N, LOUIS, ELAN D · 2011 to 2019
$7.4MPathogenesis Of Essential Tremor: Cerebellar MetabolismR01NS042859 · NINDS · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI LOUIS, ELAN D · 2003 to 2012
$4.9MPATHOLOG-OMICS - ESSENTIAL TREMOR IN THE BROADER CONTEXT OF NEURODEGENERATIONR01NS088257 · NINDS · YALE UNIVERSITY · PI FAUST, PHYLLIS L, LOUIS, ELAN D · 2015 to 2019
$3.3Min Vivo Quantification of Cerebellar GABA and NAA in Essential TremorR01NS085136 · NINDS · YALE UNIVERSITY · PI MATUSKEY, DAVID A · 2013 to 2017
$3.3MThe role of glucocerebrosidase and chaperone_mediated autophagy in Parkinsons disK08NS083738 · NINDS · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI KUO, SHENG-HAN · 2013 to 2017
$940kMitochondrial dysfunction due to aberrant mTOR-regulated mitophagy in autismK01MH096956 · NIMH · COLUMBIA UNIVERSITY HEALTH SCIENCES · PI TANG, GUOMEI · 2013 to 2016
$734kNCATS NIH HHS UL1 TR001863NICHD NIH HHS P01 HD080642NIEHS NIH HHS P30 ES009089NIMH NIH HHS K01 MH096956NINDS NIH HHS K08 NS083738NINDS NIH HHS R01 NS039422NINDS NIH HHS R01 NS042859NINDS NIH HHS R01 NS073872NINDS NIH HHS R01 NS085136NINDS NIH HHS R01 NS086736NINDS NIH HHS R01 NS088257
Abstract
In familial and sporadic multiple system atrophy (MSA) patients, deficiency of coenzyme Q10 (CoQ10) has been associated with mutations in COQ2, which encodes the second enzyme in the CoQ10 biosynthetic pathway. Cerebellar ataxia is the most common presentation of CoQ10 deficiency, suggesting that the cerebellum might be selectively vulnerable to low levels of CoQ10 To investigate whether CoQ10 deficiency represents a common feature in the brains of MSA patients independent of the presence of COQ2 mutations, we studied CoQ10 levels in postmortem brains of 12 MSA, 9 Parkinson disease (PD), 9 essential tremor (ET) patients, and 12 controls. We also assessed mitochondrial respiratory chain enzyme activities, oxidative stress, mitochondrial mass, and levels of enzymes involved in CoQ biosynthesis. Our studies revealed CoQ10 deficiency in MSA cerebellum, which was associated with impaired CoQ biosynthesis and increased oxidative stress in the absence of COQ2 mutations. The levels of CoQ10 in the cerebella of ET and PD patients were comparable or higher than in controls. These findings suggest that CoQ10 deficiency may contribute to the pathogenesis of MSA. Because no disease modifying therapies are currently available, increasing CoQ10 levels by supplementation or upregulation of its biosynthesis may represent a novel treatment strategy for MSA patients.
Indexed as
AgedAged, 80 and overAtaxiaCase-Control StudiesCerebellumFemaleHumansMaleMiddle AgedMitochondrial DiseasesMultiple System AtrophyMuscle WeaknessOxidative StressUbiquinonecoenzyme Q10UbiquinoneCerebellar ataxiaCoenzyme Q10Multiple system atrophyOxidative stress
Identifiers
What OpenQuestion holds
Textmetadata
Read underepoch 390
Registered trials
None linked
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.