Published in Methods Cell Biol on January 01, 2007
Retracted Activation of the PPAR/PGC-1alpha pathway prevents a bioenergetic deficit and effectively improves a mitochondrial myopathy phenotype. Cell Metab (2008) 2.80
Cyclic AMP produced inside mitochondria regulates oxidative phosphorylation. Cell Metab (2009) 2.45
Assessment of mitochondrial respiratory chain enzymatic activities on tissues and cultured cells. Nat Protoc (2012) 2.12
The age lipid A2E and mitochondrial dysfunction synergistically impair phagocytosis by retinal pigment epithelial cells. J Biol Chem (2008) 1.61
Mitochondrial respiratory-chain adaptations in macrophages contribute to antibacterial host defense. Nat Immunol (2016) 1.52
CHCM1/CHCHD6, novel mitochondrial protein linked to regulation of mitofilin and mitochondrial cristae morphology. J Biol Chem (2012) 1.35
Retracted mTERF2 regulates oxidative phosphorylation by modulating mtDNA transcription. Cell Metab (2009) 1.31
Retracted Endurance exercise is protective for mice with mitochondrial myopathy. J Appl Physiol (1985) (2009) 1.29
Pink1 regulates the oxidative phosphorylation machinery via mitochondrial fission. Proc Natl Acad Sci U S A (2011) 1.24
Persistent disruption of mitochondrial homeostasis after acute kidney injury. Am J Physiol Renal Physiol (2011) 1.23
Modulation of mitochondrial protein phosphorylation by soluble adenylyl cyclase ameliorates cytochrome oxidase defects. EMBO Mol Med (2009) 1.17
Pathophysiology and fate of hepatocytes in a mouse model of mitochondrial hepatopathies. Gut (2007) 1.09
The Mitochondrial Calcium Uniporter Selectively Matches Metabolic Output to Acute Contractile Stress in the Heart. Cell Rep (2015) 1.07
A phosphodiesterase 2A isoform localized to mitochondria regulates respiration. J Biol Chem (2011) 1.07
Genetic deletion of the mitochondrial phosphate carrier desensitizes the mitochondrial permeability transition pore and causes cardiomyopathy. Cell Death Differ (2014) 1.05
Mitochondrial DNA background modifies the bioenergetics of NARP/MILS ATP6 mutant cells. Hum Mol Genet (2009) 1.02
ROS-triggered phosphorylation of complex II by Fgr kinase regulates cellular adaptation to fuel use. Cell Metab (2014) 1.00
Targeting mitochondrial cardiolipin and the cytochrome c/cardiolipin complex to promote electron transport and optimize mitochondrial ATP synthesis. Br J Pharmacol (2014) 0.98
Control of oxidative phosphorylation by vitamin A illuminates a fundamental role in mitochondrial energy homoeostasis. FASEB J (2009) 0.97
AMPK activation with glabridin ameliorates adiposity and lipid dysregulation in obesity. J Lipid Res (2012) 0.94
In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space. J Neurosci (2011) 0.94
Regulation of intermediary metabolism by the PKCdelta signalosome in mitochondria. FASEB J (2010) 0.91
Inhibition of human peptide deformylase disrupts mitochondrial function. Mol Cell Biol (2010) 0.91
Glucagon regulation of oxidative phosphorylation requires an increase in matrix adenine nucleotide content through Ca2+ activation of the mitochondrial ATP-Mg/Pi carrier SCaMC-3. J Biol Chem (2013) 0.90
A metabolic shift induced by a PPAR panagonist markedly reduces the effects of pathogenic mitochondrial tRNA mutations. J Cell Mol Med (2011) 0.86
DJ-1 null dopaminergic neuronal cells exhibit defects in mitochondrial function and structure: involvement of mitochondrial complex I assembly. PLoS One (2012) 0.86
Pre-symptomatic activation of antioxidant responses and alterations in glucose and pyruvate metabolism in Niemann-Pick Type C1-deficient murine brain. PLoS One (2013) 0.82
Novel role of ATPase subunit C targeting peptides beyond mitochondrial protein import. Mol Biol Cell (2009) 0.82
An EMMPRIN-γ-catenin-Nm23 complex drives ATP production and actomyosin contractility at endothelial junctions. J Cell Sci (2014) 0.81
Mitochondrial function in human neuroblastoma cells is up-regulated and protected by NQO1, a plasma membrane redox enzyme. PLoS One (2013) 0.80
CDK4-mediated MnSOD activation and mitochondrial homeostasis in radioadaptive protection. Free Radic Biol Med (2015) 0.80
Two protein kinase C isoforms, δ and ε, regulate energy homeostasis in mitochondria by transmitting opposing signals to the pyruvate dehydrogenase complex. FASEB J (2012) 0.80
Nuclear expression of a mitochondrial DNA gene: mitochondrial targeting of allotopically expressed mutant ATP6 in transgenic mice. J Biomed Biotechnol (2012) 0.77
Knockout of Drosophila RNase ZL impairs mitochondrial transcript processing, respiration and cell cycle progression. Nucleic Acids Res (2015) 0.76
Cytochrome b5 reductase, a plasma membrane redox enzyme, protects neuronal cells against metabolic and oxidative stress through maintaining redox state and bioenergetics. Age (Dordr) (2015) 0.75
Disrupted in schizophrenia 1 (DISC1) is a constituent of the mammalian mitochondrial contact site and cristae organizing system (MICOS) complex, and is essential for oxidative phosphorylation. Hum Mol Genet (2016) 0.75
CDK1 Enhances Mitochondrial Bioenergetics for Radiation-Induced DNA Repair. Cell Rep (2015) 0.75
Elevated Energy Production in Chronic Fatigue Syndrome Patients. J Nat Sci (2016) 0.75
Intracellular Assessment of ATP Levels in Caenorhabditis elegans. Bio Protoc (2016) 0.75
Biological Membrane-Packed Mesenchymal Stem Cells Treat Acute Kidney Disease by Ameliorating Mitochondrial-Related Apoptosis. Sci Rep (2017) 0.75
Dopamine neurons derived from human ES cells efficiently engraft in animal models of Parkinson's disease. Nature (2011) 7.90
Functional engraftment of human ES cell-derived dopaminergic neurons enriched by coculture with telomerase-immortalized midbrain astrocytes. Nat Med (2006) 7.21
Mitochondrial pathology and muscle and dopaminergic neuron degeneration caused by inactivation of Drosophila Pink1 is rescued by Parkin. Proc Natl Acad Sci U S A (2006) 5.78
Pink1 regulates mitochondrial dynamics through interaction with the fission/fusion machinery. Proc Natl Acad Sci U S A (2008) 4.97
Genetic or pharmacological iron chelation prevents MPTP-induced neurotoxicity in vivo: a novel therapy for Parkinson's disease. Neuron (2003) 2.80
Mutated human SOD1 causes dysfunction of oxidative phosphorylation in mitochondria of transgenic mice. J Biol Chem (2002) 2.68
Inactivation of Drosophila DJ-1 leads to impairments of oxidative stress response and phosphatidylinositol 3-kinase/Akt signaling. Proc Natl Acad Sci U S A (2005) 2.58
Cyclic AMP produced inside mitochondria regulates oxidative phosphorylation. Cell Metab (2009) 2.45
Rescue of a mitochondrial deficiency causing Leber Hereditary Optic Neuropathy. Ann Neurol (2002) 2.13
Parkin overexpression selects against a deleterious mtDNA mutation in heteroplasmic cybrid cells. Proc Natl Acad Sci U S A (2010) 2.10
Sensitivity to oxidative stress in DJ-1-deficient dopamine neurons: an ES- derived cell model of primary Parkinsonism. PLoS Biol (2004) 2.02
Neuronal degeneration and mitochondrial dysfunction. J Clin Invest (2003) 2.00
Mitochondrial dysfunction and amyotrophic lateral sclerosis. Muscle Nerve (2006) 1.95
Dopaminergic neuronal loss, reduced neurite complexity and autophagic abnormalities in transgenic mice expressing G2019S mutant LRRK2. PLoS One (2011) 1.93
Positive contribution of pathogenic mutations in the mitochondrial genome to the promotion of cancer by prevention from apoptosis. Cancer Res (2005) 1.90
Measurements of ATP in mammalian cells. Methods (2002) 1.81
Mutant superoxide dismutase 1 forms aggregates in the brain mitochondrial matrix of amyotrophic lateral sclerosis mice. J Neurosci (2005) 1.79
Uncoupling protein-2 is critical for nigral dopamine cell survival in a mouse model of Parkinson's disease. J Neurosci (2005) 1.67
Expression profiling of Huntington's disease models suggests that brain-derived neurotrophic factor depletion plays a major role in striatal degeneration. J Neurosci (2007) 1.66
Neural mitochondrial Ca2+ capacity impairment precedes the onset of motor symptoms in G93A Cu/Zn-superoxide dismutase mutant mice. J Neurochem (2006) 1.64
Impaired PGC-1alpha function in muscle in Huntington's disease. Hum Mol Genet (2009) 1.63
The age lipid A2E and mitochondrial dysfunction synergistically impair phagocytosis by retinal pigment epithelial cells. J Biol Chem (2008) 1.61
Overexpression of CCS in G93A-SOD1 mice leads to accelerated neurological deficits with severe mitochondrial pathology. Proc Natl Acad Sci U S A (2007) 1.60
Inhibition of the leucine-rich repeat protein LINGO-1 enhances survival, structure, and function of dopaminergic neurons in Parkinson's disease models. Proc Natl Acad Sci U S A (2007) 1.58
The role of mitochondria in inherited neurodegenerative diseases. J Neurochem (2006) 1.53
Uncoupling protein 2 prevents neuronal death including that occurring during seizures: a mechanism for preconditioning. Endocrinology (2003) 1.50
Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease. Hum Mol Genet (2011) 1.44
Mitochondrial DNA and respiratory chain function in spinal cords of ALS patients. J Neurochem (2002) 1.42
The mtDNA T8993G (NARP) mutation results in an impairment of oxidative phosphorylation that can be improved by antioxidants. Hum Mol Genet (2004) 1.40
Unexpected lack of hypersensitivity in LRRK2 knock-out mice to MPTP (1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine). J Neurosci (2009) 1.36
Different regulation of wild-type and mutant Cu,Zn superoxide dismutase localization in mammalian mitochondria. Hum Mol Genet (2008) 1.35
Age-related microglial activation in 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced dopaminergic neurodegeneration in C57BL/6 mice. Brain Res (2003) 1.32
PINK1 defect causes mitochondrial dysfunction, proteasomal deficit and alpha-synuclein aggregation in cell culture models of Parkinson's disease. PLoS One (2009) 1.30
Mice lacking alpha-synuclein are resistant to mitochondrial toxins. Neurobiol Dis (2005) 1.30
Mitochondrial dysfunction and intracellular calcium dysregulation in ALS. Mech Ageing Dev (2010) 1.30
Mutant SOD1 in neuronal mitochondria causes toxicity and mitochondrial dynamics abnormalities. Hum Mol Genet (2009) 1.30
Protein phosphorylation and prevention of cytochrome oxidase inhibition by ATP: coupled mechanisms of energy metabolism regulation. Cell Metab (2011) 1.29
The mitochondrial respiratory chain is a modulator of apoptosis. J Cell Biol (2007) 1.28
The role of memantine in the treatment of psychiatric disorders other than the dementias: a review of current preclinical and clinical evidence. CNS Drugs (2012) 1.26
Cell-permeable peptide antioxidants as a novel therapeutic approach in a mouse model of amyotrophic lateral sclerosis. J Neurochem (2006) 1.26
Pink1 regulates the oxidative phosphorylation machinery via mitochondrial fission. Proc Natl Acad Sci U S A (2011) 1.24
Heterologous mitochondrial DNA recombination in human cells. Hum Mol Genet (2004) 1.23
Respiratory chain supercomplexes set the threshold for respiration defects in human mtDNA mutant cybrids. Hum Mol Genet (2006) 1.22
A pivotal role of matrix metalloproteinase-3 activity in dopaminergic neuronal degeneration via microglial activation. FASEB J (2006) 1.22
Analysis of mouse models of cytochrome c oxidase deficiency owing to mutations in Sco2. Hum Mol Genet (2010) 1.21
SOD1 targeted to the mitochondrial intermembrane space prevents motor neuropathy in the Sod1 knockout mouse. Brain (2010) 1.21
Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice. J Neurochem (2003) 1.20
Mitochondrial dynamics and bioenergetic dysfunction is associated with synaptic alterations in mutant SOD1 motor neurons. J Neurosci (2012) 1.19
Abnormal mitochondrial transport and morphology are common pathological denominators in SOD1 and TDP43 ALS mouse models. Hum Mol Genet (2013) 1.19
Mice deficient in dihydrolipoamide dehydrogenase show increased vulnerability to MPTP, malonate and 3-nitropropionic acid neurotoxicity. J Neurochem (2004) 1.19
Modulation of mitochondrial protein phosphorylation by soluble adenylyl cyclase ameliorates cytochrome oxidase defects. EMBO Mol Med (2009) 1.17
Mechanisms, models and biomarkers in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener (2013) 1.16
Bioenergetic abnormalities in discrete cerebral motor pathways presage spinal cord pathology in the G93A SOD1 mouse model of ALS. Neurobiol Dis (2006) 1.15
MTG1 codes for a conserved protein required for mitochondrial translation. Mol Biol Cell (2003) 1.15
Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation. Hum Mol Genet (2010) 1.14
Import, maturation, and function of SOD1 and its copper chaperone CCS in the mitochondrial intermembrane space. Antioxid Redox Signal (2010) 1.12
Mitochondrial function, morphology, and axonal transport in amyotrophic lateral sclerosis. Antioxid Redox Signal (2009) 1.12
Therapeutic effects of coenzyme Q10 (CoQ10) and reduced CoQ10 in the MPTP model of Parkinsonism. J Neurochem (2007) 1.10
Targeting Nrf2-mediated gene transcription by extremely potent synthetic triterpenoids attenuate dopaminergic neurotoxicity in the MPTP mouse model of Parkinson's disease. Antioxid Redox Signal (2012) 1.09
A chemical enucleation method for the transfer of mitochondrial DNA to rho(o) cells. Nucleic Acids Res (2003) 1.08
Neuroprotective effects of compounds with antioxidant and anti-inflammatory properties in a Drosophila model of Parkinson's disease. BMC Neurosci (2009) 1.08
Neuroprotective effects of synaptic modulation in Huntington's disease R6/2 mice. J Neurosci (2007) 1.07
A phosphodiesterase 2A isoform localized to mitochondria regulates respiration. J Biol Chem (2011) 1.07
cAMP and mitochondria. Physiology (Bethesda) (2013) 1.07
An assembled complex IV maintains the stability and activity of complex I in mammalian mitochondria. J Biol Chem (2007) 1.07
Muscle choline kinase beta defect causes mitochondrial dysfunction and increased mitophagy. Hum Mol Genet (2011) 1.06
Barth syndrome: cellular compensation of mitochondrial dysfunction and apoptosis inhibition due to changes in cardiolipin remodeling linked to tafazzin (TAZ) gene mutation. Biochim Biophys Acta (2013) 1.06
Enhanced ROS production and antioxidant defenses in cybrids harbouring mutations in mtDNA. Neurosci Lett (2005) 1.04
Inhibition of prolyl hydroxylase protects against 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-induced neurotoxicity: model for the potential involvement of the hypoxia-inducible factor pathway in Parkinson disease. J Biol Chem (2009) 1.03
New insights into the bioenergetics of mitochondrial disorders using intracellular ATP reporters. Mol Biol Cell (2003) 1.02
Neuroprotective effects of phenylbutyrate against MPTP neurotoxicity. Neuromolecular Med (2004) 1.01
A new method for analysis of mitochondrial DNA point mutations and assess levels of heteroplasmy. Biochem Biophys Res Commun (2006) 1.00
Periventricular white matter hyperintensities as predictors of suicide attempts in bipolar disorders and unipolar depression. Prog Neuropsychopharmacol Biol Psychiatry (2008) 1.00
Lysyl-tRNA synthetase is a target for mutant SOD1 toxicity in mitochondria. J Biol Chem (2008) 0.98
Involvement of inflammation-related miR-155 and miR-146a in diabetic nephropathy: implications for glomerular endothelial injury. BMC Nephrol (2014) 0.97
Control of oxidative phosphorylation by vitamin A illuminates a fundamental role in mitochondrial energy homoeostasis. FASEB J (2009) 0.97
Conditional transgenic mice expressing C-terminally truncated human alpha-synuclein (alphaSyn119) exhibit reduced striatal dopamine without loss of nigrostriatal pathway dopaminergic neurons. Mol Neurodegener (2009) 0.97
Dopamine transmission in DYT1 dystonia. Adv Neurol (2004) 0.96
Neurotoxicity and behavioral deficits associated with Septin 5 accumulation in dopaminergic neurons. J Neurochem (2005) 0.96
Risk factors for the prognosis of acute kidney injury under the Acute Kidney Injury Network definition: a retrospective, multicenter study in critically ill patients. Nephrology (Carlton) (2012) 0.94
In vivo pathogenic role of mutant SOD1 localized in the mitochondrial intermembrane space. J Neurosci (2011) 0.94
Additive neuroprotective effects of creatine and a cyclooxygenase 2 inhibitor against dopamine depletion in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) mouse model of Parkinson's disease. J Mol Neurosci (2003) 0.92
Regulation of intermediary metabolism by the PKCdelta signalosome in mitochondria. FASEB J (2010) 0.91
Luciferase expression for ATP imaging: application to cardiac myocytes. Methods Cell Biol (2007) 0.91
Inhibition of human peptide deformylase disrupts mitochondrial function. Mol Cell Biol (2010) 0.91
Sequence analysis of the entire mitochondrial genome in Parkinson's disease. Biochem Biophys Res Commun (2002) 0.91
Behavioral deficit, oxidative stress, and mitochondrial dysfunction precede tau pathology in P301S transgenic mice. FASEB J (2011) 0.90
A neuroprotective role of the human uncoupling protein 2 (hUCP2) in a Drosophila Parkinson's disease model. Neurobiol Dis (2012) 0.89
A kinetic assay of mitochondrial ADP-ATP exchange rate in permeabilized cells. Anal Biochem (2010) 0.89
Prohibitin reduces mitochondrial free radical production and protects brain cells from different injury modalities. J Neurosci (2012) 0.89
Mitochondrial permeability transition pore component cyclophilin D distinguishes nigrostriatal dopaminergic death paradigms in the MPTP mouse model of Parkinson's disease. Antioxid Redox Signal (2011) 0.89
Suicide in a large population of former psychiatric inpatients. Psychiatry Clin Neurosci (2011) 0.88
Truncated peroxisome proliferator-activated receptor-γ coactivator 1α splice variant is severely altered in Huntington's disease. Neurodegener Dis (2011) 0.88
The mitochondrial calcium regulator cyclophilin D is an essential component of oestrogen-mediated neuroprotection in amyotrophic lateral sclerosis. Brain (2012) 0.88
Command hallucinations with self-stabbing associated with zolpidem overdose. J Clin Psychiatry (2010) 0.86
adPEO mutations in ANT1 impair ADP-ATP translocation in muscle mitochondria. Hum Mol Genet (2011) 0.86
Functional neuroimaging in specific phobia. Psychiatry Res (2012) 0.86
Bezafibrate administration improves behavioral deficits and tau pathology in P301S mice. Hum Mol Genet (2012) 0.86