Published in Hum Mol Genet on March 18, 2009
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Cytochrome c oxidase is required for the assembly/stability of respiratory complex I in mouse fibroblasts. Mol Cell Biol (2006) 1.64
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Double-strand breaks of mouse muscle mtDNA promote large deletions similar to multiple mtDNA deletions in humans. Hum Mol Genet (2005) 1.56
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Retracted mTERF2 regulates oxidative phosphorylation by modulating mtDNA transcription. Cell Metab (2009) 1.31
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The mitochondrial complex I inhibitor rotenone triggers a cerebral tauopathy. J Neurochem (2005) 1.27
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The mtDNA mutation spectrum of the progeroid Polg mutator mouse includes abundant control region multimers. Cell Metab (2010) 1.16
The striatum is highly susceptible to mitochondrial oxidative phosphorylation dysfunctions. J Neurosci (2011) 1.14
A defect in the mitochondrial complex III, but not complex IV, triggers early ROS-dependent damage in defined brain regions. Hum Mol Genet (2012) 1.13
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In vivo methylation of mtDNA reveals the dynamics of protein-mtDNA interactions. Nucleic Acids Res (2009) 1.10
Cells lacking Rieske iron-sulfur protein have a reactive oxygen species-associated decrease in respiratory complexes I and IV. Mol Cell Biol (2011) 1.10
Striatal dysfunctions associated with mitochondrial DNA damage in dopaminergic neurons in a mouse model of Parkinson's disease. J Neurosci (2011) 1.09
A chemical enucleation method for the transfer of mitochondrial DNA to rho(o) cells. Nucleic Acids Res (2003) 1.08
The background of mitochondrial DNA haplogroup J increases the sensitivity of Leber's hereditary optic neuropathy cells to 2,5-hexanedione toxicity. PLoS One (2009) 1.08
PGC-1alpha/beta upregulation is associated with improved oxidative phosphorylation in cells harboring nonsense mtDNA mutations. Hum Mol Genet (2007) 1.07
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Increased mitochondrial biogenesis in muscle improves aging phenotypes in the mtDNA mutator mouse. Hum Mol Genet (2012) 1.02
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Extended polyglutamine repeats trigger a feedback loop involving the mitochondrial complex III, the proteasome and huntingtin aggregates. Hum Mol Genet (2007) 1.01
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Association of optic disc size with development and prognosis of Leber's hereditary optic neuropathy. Invest Ophthalmol Vis Sci (2008) 0.98
Lack of cytochrome c in mouse fibroblasts disrupts assembly/stability of respiratory complexes I and IV. J Biol Chem (2008) 0.98
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Respiratory complex I is essential to induce a Warburg profile in mitochondria-defective tumor cells. Cancer Metab (2013) 0.96
Rare primary mitochondrial DNA mutations and probable synergistic variants in Leber's hereditary optic neuropathy. PLoS One (2012) 0.96
An ALS mouse model with a permeable blood-brain barrier benefits from systemic cyclosporine A treatment. J Neurochem (2004) 0.96
BCL-2 improves oxidative phosphorylation and modulates adenine nucleotide translocation in mitochondria of cells harboring mutant mtDNA. J Biol Chem (2002) 0.95
A mutation threshold distinguishes the antitumorigenic effects of the mitochondrial gene MTND1, an oncojanus function. Cancer Res (2011) 0.95
Mutations in CYC1, encoding cytochrome c1 subunit of respiratory chain complex III, cause insulin-responsive hyperglycemia. Am J Hum Genet (2013) 0.94
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Mouse models of oxidative phosphorylation defects: powerful tools to study the pathobiology of mitochondrial diseases. Biochim Biophys Acta (2008) 0.90
Increases in mitochondrial biogenesis impair carcinogenesis at multiple levels. Mol Oncol (2011) 0.90
Decreased mitochondrial tRNALys steady-state levels and aminoacylation are associated with the pathogenic G8313A mitochondrial DNA mutation. Biochem J (2003) 0.89
Mitochondrial involvement in amyotrophic lateral sclerosis: trigger or target? Mol Neurobiol (2006) 0.89
Mitochondrial transcription: lessons from mouse models. Biochim Biophys Acta (2011) 0.88
An inherited mitochondrial DNA disruptive mutation shifts to homoplasmy in oncocytic tumor cells. Hum Mutat (2009) 0.87
Superoxide released into the mitochondrial matrix. Free Radic Biol Med (2006) 0.86
A metabolic shift induced by a PPAR panagonist markedly reduces the effects of pathogenic mitochondrial tRNA mutations. J Cell Mol Med (2011) 0.86
A novel in-frame 18-bp microdeletion in MT-CYB causes a multisystem disorder with prominent exercise intolerance. Hum Mutat (2014) 0.86
Overexpression of Twinkle-helicase protects cardiomyocytes from genotoxic stress caused by reactive oxygen species. Proc Natl Acad Sci U S A (2013) 0.86
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Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage. Hum Mol Genet (2013) 0.85
The role of cytochrome c oxidase deficiency in ROS and amyloid plaque formation. J Bioenerg Biomembr (2009) 0.85
A clinically complex form of dominant optic atrophy (OPA8) maps on chromosome 16. Hum Mol Genet (2011) 0.83
Cybrid studies establish the causal link between the mtDNA m.3890G>A/MT-ND1 mutation and optic atrophy with bilateral brainstem lesions. Biochim Biophys Acta (2012) 0.83
Bezafibrate improves mitochondrial function in the CNS of a mouse model of mitochondrial encephalopathy. Mitochondrion (2012) 0.82
Nitric oxide synthesis is increased in cybrid cells with m.3243A>G mutation. Int J Mol Sci (2012) 0.82
Increase in muscle mitochondrial biogenesis does not prevent muscle loss but increased tumor size in a mouse model of acute cancer-induced cachexia. PLoS One (2012) 0.82
Transient systemic mtDNA damage leads to muscle wasting by reducing the satellite cell pool. Hum Mol Genet (2013) 0.82
Mouse models of oxidative phosphorylation dysfunction and disease. Methods (2008) 0.82
The cytochrome b p.278Y>C mutation causative of a multisystem disorder enhances superoxide production and alters supramolecular interactions of respiratory chain complexes. Hum Mol Genet (2013) 0.81
Long-term bezafibrate treatment improves skin and spleen phenotypes of the mtDNA mutator mouse. PLoS One (2012) 0.81
Mouse models of Parkinson's disease associated with mitochondrial dysfunction. Mol Cell Neurosci (2012) 0.81
Regional susceptibilities to mitochondrial dysfunctions in the CNS. Biol Chem (2012) 0.80
Mitochondrial alterations during carcinogenesis: a review of metabolic transformation and targets for anticancer treatments. Adv Cancer Res (2013) 0.80
Systematic association studies of mitochondrial DNA variations in schizophrenia: focus on the ND5 gene. Schizophr Bull (2007) 0.80
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