Metabolic effects of bezafibrate in mitochondrial disease

Rewiring of Glutamine Metabolism Is a Bioenergetic Adaptation of Human Cells with Mitochondrial DNA Mutations

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Aspartate is an endogenous metabolic limitation for tumour growth

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MetaboAnalyst 4.0: towards more transparent and integrative metabolomics analysis

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Metabolomes of mitochondrial diseases and inclusion body myositis patients: treatment targets and biomarkers

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Mitochondrial genetic medicine

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Dimethyl fumarate mediates Nrf2-dependent mitochondrial biogenesis in mice and humans

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Salmon provides fast and bias-aware quantification of transcript expression

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Glutamine metabolism regulates autophagy-dependent mTORC1 reactivation during amino acid starvation

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Paradoxical Inhibition of Glycolysis by Pioglitazone Opposes the Mitochondriopathy Caused by AIF Deficiency

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The effect of percutaneous coronary intervention on habitual physical activity in older patients

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Emerging therapies for mitochondrial disorders

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Sustained AMPK activation improves muscle function in a mitochondrial myopathy mouse model by promoting muscle fiber regeneration

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FGF21 is a biomarker for mitochondrial translation and mtDNA maintenance disorders

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Prevalence of nuclear and mitochondrial DNA mutations related to adult mitochondrial disease

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Bioreactance is a reliable method for estimating cardiac output at rest and during exercise

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Gender-specific pathway differences in the human serum metabolome

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limma powers differential expression analyses for RNA-sequencing and microarray studies

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MitoCarta2.0: an updated inventory of mammalian mitochondrial proteins

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A novel immunofluorescent assay to investigate oxidative phosphorylation deficiency in mitochondrial myopathy: understanding mechanisms and improving diagnosis

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A Metabolic Signature of Mitochondrial Dysfunction Revealed through a Monogenic Form of Leigh Syndrome

(2015) Julie Thompson Legault et al.   Cell Reports

Extreme-Depth Re-sequencing of Mitochondrial DNA Finds No Evidence of Paternal Transmission in Humans

(2015) Angela Pyle et al.   PLoS Genetics

Implementing a Class of Permutation Tests: ThecoinPackage

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A Novel, Open Access Method to Assess Sleep Duration Using a Wrist-Worn Accelerometer

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NAD+-Dependent Activation of Sirt1 Corrects the Phenotype in a Mouse Model of Mitochondrial Disease

(2014) Raffaele Cerutti et al.   Cell Metabolism

An atlas of genetic influences on human blood metabolites

(2014) So-Youn Shin et al.   NATURE GENETICS

Oxidation of Alpha-Ketoglutarate Is Required for Reductive Carboxylation in Cancer Cells with Mitochondrial Defects

(2014) Andrew R. Mullen et al.   Cell Reports

Defining the action spectrum of potential PGC-1α activators on a mitochondrial and cellular level in vivo

(2013) Annette Hofer et al.   HUMAN MOLECULAR GENETICS

Elevated FGF21 secretion, PGC-1α and ketogenic enzyme expression are hallmarks of iron–sulfur cluster depletion in human skeletal muscle

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Separating Movement and Gravity Components in an Acceleration Signal and Implications for the Assessment of Human Daily Physical Activity

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Cardiomyopathy is common in patients with the mitochondrial DNA m.3243A>G mutation and correlates with mutation load

(2012) Kieren G. Hollingsworth et al.   NEUROMUSCULAR DISORDERS

Long-Term Bezafibrate Treatment Improves Skin and Spleen Phenotypes of the mtDNA Mutator Mouse

(2012) Lloye M. Dillon et al.   PLoS One

In Vivo Correction of COX Deficiency by Activation of the AMPK/PGC-1α Axis

(2011) Carlo Viscomi et al.   Cell Metabolism

Effect of bezafibrate treatment on late-onset mitochondrial myopathy in mice

(2011) Shuichi Yatsuga et al.   HUMAN MOLECULAR GENETICS

Long-Term Follow-Up of Bezafibrate Treatment in Patients With the Myopathic Form of Carnitine Palmitoyltransferase 2 Deficiency

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Ketogenic diet slows down mitochondrial myopathy progression in mice

(2010) Sofia Ahola-Erkkilä et al.   HUMAN MOLECULAR GENETICS

edgeR: a Bioconductor package for differential expression analysis of digital gene expression data

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Mapping identifiers for the integration of genomic datasets with the R/Bioconductor package biomaRt

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