Probucol ameliorates renal and metabolic sequelae of primary CoQ deficiency in Pdss2 mutant mice
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The COQ2 genotype predicts the severity of coenzyme Q10 deficiencyPrimary respiratory chain disease causes tissue-specific dysregulation of the global transcriptome and nutrient-sensing signaling networkDysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiencyNon-additive genome-wide association scan reveals a new gene associated with habitual coffee consumptionIntegrative analysis of independent transcriptome data for rare diseasesIntegrated transcriptome analysis across mitochondrial disease etiologies and tissues improves understanding of common cellular adaptations to respiratory chain dysfunction.Mitochondrial dysfunction in inherited renal disease and acute kidney injuryAn overview of current mouse models recapitulating coenzyme q10 deficiency syndromeEffects of inhibiting CoQ10 biosynthesis with 4-nitrobenzoate in human fibroblasts.Resveratrol and para-coumarate serve as ring precursors for coenzyme Q biosynthesis.Parkinson's disease-like neuromuscular defects occur in prenyl diphosphate synthase subunit 2 (Pdss2) mutant mice.Inhibiting cytosolic translation and autophagy improves health in mitochondrial disease.Restoring de novo coenzyme Q biosynthesis in Caenorhabditis elegans coq-3 mutants yields profound rescue compared to exogenous coenzyme Q supplementation.Mitochondrial function and lifespan of mice with controlled ubiquinone biosynthesisTissue-specific oxidative stress and loss of mitochondria in CoQ-deficient Pdss2 mutant mice.Focal segmental glomerulosclerosis is associated with a PDSS2 haplotype and, independently, with a decreased content of coenzyme Q10.Mitochondrial respiratory chain disease discrimination by retrospective cohort analysis of blood metabolites.ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption.Molecular genetics of ubiquinone biosynthesis in animals.A review on the role of quinones in renal disorders.Genetic bases and clinical manifestations of coenzyme Q10 (CoQ 10) deficiency.Pharmacologic modeling of primary mitochondrial respiratory chain dysfunction in zebrafish.Nutritional interventions in primary mitochondrial disorders: Developing an evidence base.Pharmacologic targeting of sirtuin and PPAR signaling improves longevity and mitochondrial physiology in respiratory chain complex I mutant Caenorhabditis elegans.Integrated Approaches to Drug Discovery for Oxidative Stress-Related Retinal Diseases.Knockdown of the coenzyme Q synthesis gene Smed-dlp1 affects planarian regeneration and tissue homeostasis.Mitochondrial respiration without ubiquinone biosynthesis.Genetics of coenzyme q10 deficiency.Probucol inhibits JAK2-STAT pathway activation and protects human glomerular mesangial cells from tert-butyl hydroperoxide induced premature senescence.Nutritional Interventions for Mitochondrial OXPHOS Deficiencies: Mechanisms and Model Systems.
P2860
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P2860
Probucol ameliorates renal and metabolic sequelae of primary CoQ deficiency in Pdss2 mutant mice
description
2011 nî lūn-bûn
@nan
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
2011年论文
@zh
2011年论文
@zh-cn
name
Probucol ameliorates renal and ...... eficiency in Pdss2 mutant mice
@ast
Probucol ameliorates renal and ...... eficiency in Pdss2 mutant mice
@en
type
label
Probucol ameliorates renal and ...... eficiency in Pdss2 mutant mice
@ast
Probucol ameliorates renal and ...... eficiency in Pdss2 mutant mice
@en
prefLabel
Probucol ameliorates renal and ...... eficiency in Pdss2 mutant mice
@ast
Probucol ameliorates renal and ...... eficiency in Pdss2 mutant mice
@en
P2093
P2860
P356
P1476
Probucol ameliorates renal and ...... eficiency in Pdss2 mutant mice
@en
P2093
Beth Marbois
Catherine F Clarke
David L Gasser
Eiko Nakamaru-Ogiso
Erzsebet Polyak
Ezinne Okwuego
Itzhak Nissim
Jia Yan Chen
Jonathan S Maltzman
Julian Ostrovsky
P2860
P304
P356
10.1002/EMMM.201100149
P50
P577
2011-06-08T00:00:00Z