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Mitochondrial ribosomes in cancer. Semin Cancer Biol (2017) 0.76

OPA1 mutations induce mitochondrial DNA instability and optic atrophy 'plus' phenotypes. Brain (2007) 3.35

Mutation of OPA1 causes dominant optic atrophy with external ophthalmoplegia, ataxia, deafness and multiple mitochondrial DNA deletions: a novel disorder of mtDNA maintenance. Brain (2007) 3.14

The MFN2 gene is responsible for mitochondrial DNA instability and optic atrophy 'plus' phenotype. Brain (2011) 3.04

Adenine nucleotide translocase 2 is a key mitochondrial protein in cancer metabolism. Biochim Biophys Acta (2010) 1.79

Identification of a gene expression profile associated with operational tolerance among a selected group of stable kidney transplant patients. Transpl Int (2011) 1.73

A chorionic gonadotropin-sensitive mutation in the follicle-stimulating hormone receptor as a cause of familial gestational spontaneous ovarian hyperstimulation syndrome. N Engl J Med (2003) 1.69

Molecular screening of 980 cases of suspected hereditary optic neuropathy with a report on 77 novel OPA1 mutations. Hum Mutat (2009) 1.67

Heterozygous OPA1 mutations in Behr syndrome. Brain (2010) 1.57

eOPA1: an online database for OPA1 mutations. Hum Mutat (2005) 1.43

OPA1-associated disorders: phenotypes and pathophysiology. Int J Biochem Cell Biol (2009) 1.40

Mitochondrial dynamics and disease, OPA1. Biochim Biophys Acta (2006) 1.35

Two-step differential expression analysis reveals a new set of genes involved in thyroid oncocytic tumors. J Clin Endocrinol Metab (2004) 1.35

Hereditary optic neuropathies share a common mitochondrial coupling defect. Ann Neurol (2008) 1.33

Effects of OPA1 mutations on mitochondrial morphology and apoptosis: relevance to ADOA pathogenesis. J Cell Physiol (2007) 1.30

Structure and chromosomal distribution of human mitochondrial pseudogenes. Genomics (2002) 1.27

ANT2 isoform required for cancer cell glycolysis. J Bioenerg Biomembr (2005) 1.26

Estrogen-related receptor alpha and PGC-1-related coactivator constitute a novel complex mediating the biogenesis of functional mitochondria. FEBS J (2010) 1.20

OPA1 functions in mitochondria and dysfunctions in optic nerve. Int J Biochem Cell Biol (2009) 1.19

Transcriptional profiling reveals coordinated up-regulation of oxidative metabolism genes in thyroid oncocytic tumors. J Clin Endocrinol Metab (2004) 1.19

OPA1 R445H mutation in optic atrophy associated with sensorineural deafness. Ann Neurol (2005) 1.18

OPA1 links human mitochondrial genome maintenance to mtDNA replication and distribution. Genome Res (2010) 1.17

Leigh-like encephalopathy complicating Leber's hereditary optic neuropathy. Ann Neurol (2002) 1.14

PGC-1-related coactivator and targets are upregulated in thyroid oncocytoma. Biochem Biophys Res Commun (2003) 1.14

Mitochondrial coupling defect in Charcot-Marie-Tooth type 2A disease. Ann Neurol (2007) 1.13

Mitochondrial dysfunction and pathophysiology of Charcot-Marie-Tooth disease involving GDAP1 mutations. Exp Neurol (2010) 1.12

Dominant optic atrophy. Orphanet J Rare Dis (2012) 1.11

Whole mitochondrial genome screening in maternally inherited non-syndromic hearing impairment using a microarray resequencing mitochondrial DNA chip. Eur J Hum Genet (2007) 1.10

Maternal smoking is associated with mitochondrial DNA depletion and respiratory chain complex III deficiency in placenta. Am J Physiol Endocrinol Metab (2005) 1.03

The human OPA1delTTAG mutation induces premature age-related systemic neurodegeneration in mouse. Brain (2012) 1.03

MADGene: retrieval and processing of gene identifier lists for the analysis of heterogeneous microarray datasets. Bioinformatics (2011) 1.01

Estrogen-related receptor alpha modulates lactate dehydrogenase activity in thyroid tumors. PLoS One (2013) 1.00

Fourteen novel OPA1 mutations in autosomal dominant optic atrophy including two de novo mutations in sporadic optic atrophy. Hum Mutat (2003) 0.99

Resveratrol induces a mitochondrial complex I-dependent increase in NADH oxidation responsible for sirtuin activation in liver cells. J Biol Chem (2013) 0.98

Low serum testosterone assayed by liquid chromatography-tandem mass spectrometry. Comparison with five immunoassay techniques. Clin Chim Acta (2007) 0.97

Dynamic regulation of mitochondrial network and oxidative functions during 3T3-L1 fat cell differentiation. J Physiol Biochem (2011) 0.97

Mitochondrial DNA in the oocyte and the developing embryo. Curr Top Dev Biol (2007) 0.97

Rare primary mitochondrial DNA mutations and probable synergistic variants in Leber's hereditary optic neuropathy. PLoS One (2012) 0.96

Comparison of spheroids formed by rat glioma stem cells and neural stem cells reveals differences in glucose metabolism and promising therapeutic applications. J Biol Chem (2012) 0.96

Increase of mitochondrial DNA content and transcripts in early bovine embryogenesis associated with upregulation of mtTFA and NRF1 transcription factors. Reprod Biol Endocrinol (2005) 0.94

Quantitative two-dimensional HRMAS 1H-NMR spectroscopy-based metabolite profiling of human cancer cell lines and response to chemotherapy. Magn Reson Med (2010) 0.93

Reversible optic neuropathy with OPA1 exon 5b mutation. Ann Neurol (2008) 0.93

Experimental determination of organelle targeting-peptide cleavage sites using transient expression of green fluorescent protein translational fusions. Anal Biochem (2012) 0.92

ANT2 expression under hypoxic conditions produces opposite cell-cycle behavior in 143B and HepG2 cancer cells. Mol Carcinog (2005) 0.92

Dinitrophenol-induced mitochondrial uncoupling in vivo triggers respiratory adaptation in HepG2 cells. Biochim Biophys Acta (2005) 0.92

Immune response and mitochondrial metabolism are commonly deregulated in DMD and aging skeletal muscle. PLoS One (2011) 0.92

Effects of the cannabinoid CB1 antagonist rimonabant on hepatic mitochondrial function in rats fed a high-fat diet. Am J Physiol Endocrinol Metab (2009) 0.92

Papilloedema and MRI enhancement of the prechiasmal optic nerve at the acute stage of Leber hereditary optic neuropathy. J Neurol Neurosurg Psychiatry (2010) 0.91

Abnormal stimulation of the thyrotrophin receptor during gestation. Hum Reprod Update (2004) 0.91

Gene profiling reveals specific oncogenic mechanisms and signaling pathways in oncocytic and papillary thyroid carcinoma. Oncogene (2005) 0.91

Random mtDNA deletions and functional consequence in aged human skeletal muscle. Biochem Biophys Res Commun (2005) 0.91

The association of autosomal dominant optic atrophy and moderate deafness may be due to the R445H mutation in the OPA1 gene. Am J Ophthalmol (2003) 0.90

Bioenergetic defect associated with mKATP channel opening in a mouse model carrying a mitofusin 2 mutation. FASEB J (2011) 0.89

Diagnosis of myotubular myopathy in the oldest known manifesting female carrier: a clinical and genetic study. Neuromuscul Disord (2007) 0.89

Genetically determined optic neuropathies. Curr Opin Neurol (2010) 0.89

Mitochondrial complex I deficiency in GDAP1-related autosomal dominant Charcot-Marie-Tooth disease (CMT2K). Neurogenetics (2008) 0.89

Mitochondrial bioenergetic background confers a survival advantage to HepG2 cells in response to chemotherapy. Mol Carcinog (2009) 0.89

Metabolically induced heteroplasmy shifting and l-arginine treatment reduce the energetic defect in a neuronal-like model of MELAS. Biochim Biophys Acta (2012) 0.89

Hereditary spastic paraplegia-like disorder due to a mitochondrial ATP6 gene point mutation. Mitochondrion (2010) 0.88

Adenine nucleotide translocator promotes oxidative phosphorylation and mild uncoupling in mitochondria after dexamethasone treatment. Am J Physiol Endocrinol Metab (2007) 0.88

Comparison of buccal and blood-derived canine DNA, either native or whole genome amplified, for array-based genome-wide association studies. BMC Res Notes (2011) 0.87

MFN2, a new gene responsible for mitochondrial DNA depletion. Brain (2012) 0.87

Prevalence of rare mitochondrial DNA mutations in mitochondrial disorders. J Med Genet (2013) 0.87

PGC-1-related coactivator modulates mitochondrial-nuclear crosstalk through endogenous nitric oxide in a cellular model of oncocytic thyroid tumours. PLoS One (2009) 0.87

Relative frequencies of inherited retinal dystrophies and optic neuropathies in Southern France: assessment of 21-year data management. Ophthalmic Epidemiol (2013) 0.86