ArticleEMBO molecular medicine2011
Probucol ameliorates renal and metabolic sequelae of primary CoQ deficiency in Pdss2 mutant mice.
Article in EMBO molecular medicine, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers.
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Who cites it
34 citing papers in PubMed, 48 citations in OpenAlex.
- Understanding coenzyme Q.Physiological reviews · 2024Review
- Mitochondrial disease and epilepsy in children.Frontiers in neurology · 2024Review
- Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings.Antioxidants (Basel, Switzerland) · 2021Review
- The pursuit of precision mitochondrial medicine: Harnessing preclinical cellular and animal models to optimize mitochondrial disease therapeutic discovery.Journal of inherited metabolic disease · 2021Review
- Depletion of essential isoprenoids and ER stress induction following acute liver-specific deletion of HMG-CoA reductase.Journal of lipid research · 2020Article
- Vanillic Acid Restores Coenzyme Q Biosynthesis and ATP Production in Human Cells LackingOxidative medicine and cellular longevity · 2019Article
- CoQBiochimica et biophysica acta. Molecular basis of disease · 2018Article
- Pharmacologic modeling of primary mitochondrial respiratory chain dysfunction in zebrafish.Neurochemistry international · 2018Article
- Nutritional Interventions for Mitochondrial OXPHOS Deficiencies: Mechanisms and Model Systems.Annual review of pathology · 2018Review
- Nutritional interventions in primary mitochondrial disorders: Developing an evidence base.Molecular genetics and metabolism · 2016Review
- Non-additive genome-wide association scan reveals a new gene associated with habitual coffee consumption.Scientific reports · 2016Article
- Mitochondrial dysfunction in inherited renal disease and acute kidney injury.Nature reviews. Nephrology · 2016Review
- Integrated Approaches to Drug Discovery for Oxidative Stress-Related Retinal Diseases.Oxidative medicine and cellular longevity · 2016Review
- Knockdown of the coenzyme Q synthesis gene Smed-dlp1 affects planarian regeneration and tissue homeostasis.Redox biology · 2015Article
- Inhibiting cytosolic translation and autophagy improves health in mitochondrial disease.Human molecular genetics · 2015Article
- Pharmacologic targeting of sirtuin and PPAR signaling improves longevity and mitochondrial physiology in respiratory chain complex I mutant Caenorhabditis elegans.Mitochondrion · 2015Article
- Resveratrol and para-coumarate serve as ring precursors for coenzyme Q biosynthesis.Journal of lipid research · 2015Article
- Mitochondrial function and lifespan of mice with controlled ubiquinone biosynthesis.Nature communications · 2015Article
- Genetic bases and clinical manifestations of coenzyme Q10 (CoQ 10) deficiency.Journal of inherited metabolic disease · 2015Review
- Integrative analysis of independent transcriptome data for rare diseases.Methods (San Diego, Calif.) · 2014Article
Corrections and comments
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Authors and funding
16 authors at 3 institutions in 1 country.
Funding
Abstract
Therapy of mitochondrial respiratory chain diseases is complicated by limited understanding of cellular mechanisms that cause the widely variable clinical findings. Here, we show that focal segmental glomerulopathy-like kidney disease in Pdss2 mutant animals with primary coenzyme Q (CoQ) deficiency is significantly ameliorated by oral treatment with probucol (1% w/w). Preventative effects in missense mutant mice are similar whether fed probucol from weaning or for 3 weeks prior to typical nephritis onset. Furthermore, treating symptomatic animals for 2 weeks with probucol significantly reduces albuminuria. Probucol has a more pronounced health benefit than high-dose CoQ(10) supplementation and uniquely restores CoQ(9) content in mutant kidney. Probucol substantially mitigates transcriptional alterations across many intermediary metabolic domains, including peroxisome proliferator-activated receptor (PPAR) pathway signaling. Probucol's beneficial effects on the renal and metabolic manifestations of Pdss2 disease occur despite modest induction of oxidant stress and appear independent of its hypolipidemic effects. Rather, decreased CoQ(9) content and altered PPAR pathway signaling appear, respectively, to orchestrate the glomerular and global metabolic consequences of primary CoQ deficiency, which are both preventable and treatable with oral probucol therapy.
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Registered trials
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.