Published in Biochim Biophys Acta on September 01, 2003
Tissue-specific cytochrome c oxidase assembly defects due to mutations in SCO2 and SURF1. Biochem J (2005) 1.23
YME1L controls the accumulation of respiratory chain subunits and is required for apoptotic resistance, cristae morphogenesis, and cell proliferation. Mol Biol Cell (2012) 1.10
Diminished synthesis of subunit a (ATP6) and altered function of ATP synthase and cytochrome c oxidase due to the mtDNA 2 bp microdeletion of TA at positions 9205 and 9206. Biochem J (2004) 0.92
Light and electron microscopy characteristics of the muscle of patients with SURF1 gene mutations associated with Leigh disease. J Clin Pathol (2007) 0.83
Mitochondrial vulnerability and increased susceptibility to nutrient-induced cytotoxicity in fibroblasts from leigh syndrome French canadian patients. PLoS One (2015) 0.82
Tissue- and species-specific differences in cytochrome c oxidase assembly induced by SURF1 defects. Biochim Biophys Acta (2016) 0.82
CD36 overexpression predisposes to arrhythmias but reduces infarct size in spontaneously hypertensive rats: gene expression profile analysis. Physiol Genomics (2011) 0.79
Interferons, signal transduction pathways, and the central nervous system. J Interferon Cytokine Res (2014) 0.78
Data on cytochrome c oxidase assembly in mice and human fibroblasts or tissues induced by SURF1 defect. Data Brief (2016) 0.75
Noninvasive diagnostics of mitochondrial disorders in isolated lymphocytes with high resolution respirometry. BBA Clin (2014) 0.75
Cyanide inhibition and pyruvate-induced recovery of cytochrome c oxidase. J Bioenerg Biomembr (2010) 0.75
Low mitochondrial respiratory chain content correlates with tumor aggressiveness in renal cell carcinoma. Carcinogenesis (2002) 2.04
Significance of respirasomes for the assembly/stability of human respiratory chain complex I. J Biol Chem (2004) 1.68
TMEM70 mutations cause isolated ATP synthase deficiency and neonatal mitochondrial encephalocardiomyopathy. Nat Genet (2008) 1.45
Clonal expansion of mutated mitochondrial DNA is associated with tumor formation and complex I deficiency in the benign renal oncocytoma. Hum Mol Genet (2007) 1.27
Tissue-specific cytochrome c oxidase assembly defects due to mutations in SCO2 and SURF1. Biochem J (2005) 1.23
Mitochondrial ATP synthase deficiency due to a mutation in the ATP5E gene for the F1 epsilon subunit. Hum Mol Genet (2010) 1.18
Leigh-like encephalopathy complicating Leber's hereditary optic neuropathy. Ann Neurol (2002) 1.14
Mitochondrial diseases and genetic defects of ATP synthase. Biochim Biophys Acta (2006) 1.14
Glycerophosphate-dependent hydrogen peroxide production by brown adipose tissue mitochondria and its activation by ferricyanide. J Bioenerg Biomembr (2002) 1.14
YME1L controls the accumulation of respiratory chain subunits and is required for apoptotic resistance, cristae morphogenesis, and cell proliferation. Mol Biol Cell (2012) 1.10
Retrospective, multicentric study of 180 children with cytochrome C oxidase deficiency. Pediatr Res (2005) 1.10
Direct linkage of mitochondrial genome variation to risk factors for type 2 diabetes in conplastic strains. Genome Res (2007) 1.10
Reduced respiratory control with ADP and changed pattern of respiratory chain enzymes as a result of selective deficiency of the mitochondrial ATP synthase. Pediatr Res (2004) 1.08
Mitochondria and reactive oxygen species in renal cancer. Biochimie (2007) 1.08
A new role for the von Hippel-Lindau tumor suppressor protein: stimulation of mitochondrial oxidative phosphorylation complex biogenesis. Carcinogenesis (2004) 1.07
Induction of muscle thermogenesis by high-fat diet in mice: association with obesity-resistance. Am J Physiol Endocrinol Metab (2008) 1.04
HIF and reactive oxygen species regulate oxidative phosphorylation in cancer. Carcinogenesis (2008) 1.03
Evaluation of mitochondrial membrane potential using a computerized device with a tetraphenylphosphonium-selective electrode. Anal Biochem (2006) 1.01
Mutations in TMEM76* cause mucopolysaccharidosis IIIC (Sanfilippo C syndrome). Am J Hum Genet (2006) 1.01
Occurrence of A-kinase anchor protein and associated cAMP-dependent protein kinase in the inner compartment of mammalian mitochondria. FEBS Lett (2006) 1.00
Mitochondrial complex I is deficient in renal oncocytomas. Carcinogenesis (2003) 0.99
Mitochondrial ATP synthase levels in brown adipose tissue are governed by the c-Fo subunit P1 isoform. FASEB J (2007) 0.95
Ngoye virus: a novel evolutionary lineage within the genus Flavivirus. J Gen Virol (2006) 0.94
Diminished synthesis of subunit a (ATP6) and altered function of ATP synthase and cytochrome c oxidase due to the mtDNA 2 bp microdeletion of TA at positions 9205 and 9206. Biochem J (2004) 0.92
Effect of metformin therapy on cardiac function and survival in a volume-overload model of heart failure in rats. Clin Sci (Lond) (2011) 0.92
cblE type of homocystinuria due to methionine synthase reductase deficiency: functional correction by minigene expression. Hum Mutat (2005) 0.92
Knockdown of F1 epsilon subunit decreases mitochondrial content of ATP synthase and leads to accumulation of subunit c. Biochim Biophys Acta (2009) 0.91
Natural antisense transcripts of HIF-1alpha are conserved in rodents. Gene (2004) 0.90
Inhibition of cytochrome c oxidase subunit 4 precursor processing by the hypoxia mimic cobalt chloride. Biochem Biophys Res Commun (2006) 0.90
Mitochondrial disorders in renal tumors. Mitochondrion (2006) 0.89
Contiguous X-chromosome deletion syndrome encompassing the BTK, TIMM8A, TAF7L, and DRP2 genes. J Clin Immunol (2007) 0.87
Comparison of the effect of mitochondrial inhibitors on mitochondrial membrane potential in two different cell lines using flow cytometry and spectrofluorometry. Cytometry A (2003) 0.86
High efficiency of ROS production by glycerophosphate dehydrogenase in mammalian mitochondria. Arch Biochem Biophys (2008) 0.86
Inhibition of glycerophosphate-dependent H2O2 generation in brown fat mitochondria by idebenone. Biochem Biophys Res Commun (2005) 0.86
IL-1 and LPS but not IL-6 inhibit differentiation and downregulate PPAR gamma in brown adipocytes. Cytokine (2004) 0.86
Decreased affinity for oxygen of cytochrome-c oxidase in Leigh syndrome caused by SURF1 mutations. Am J Physiol Cell Physiol (2004) 0.86
Mitochondrial encephalocardio-myopathy with early neonatal onset due to TMEM70 mutation. Arch Dis Child (2010) 0.83
Neonatal cardiac mitochondria and ischemia/reperfusion injury. Mol Cell Biochem (2009) 0.83
New evidence of a mitochondrial genetic background paradox: impact of the J haplogroup on the A3243G mutation. BMC Med Genet (2008) 0.82
Mitochondrially targeted α-tocopheryl succinate is antiangiogenic: potential benefit against tumor angiogenesis but caution against wound healing. Antioxid Redox Signal (2011) 0.82
Mechanisms participating in oxidative damage of isolated rat hepatocytes. Arch Toxicol (2008) 0.82
[Clinical, biochemical and molecular characteristics in 11 Czech children with tyrosinemia type I]. Cas Lek Cesk (2010) 0.81
Assembly factors of F1FO-ATP synthase across genomes. Proteins (2005) 0.81
Mitochondrial complex I inhibition in cerebral cortex of immature rats following homocysteic acid-induced seizures. Exp Neurol (2006) 0.81
A sequence predicted to form a stem-loop is proposed to be required for formation of an RNA-protein complex involving the 3'UTR of beta-subunit F0F1-ATPase mRNA. Biochim Biophys Acta (2008) 0.81
Detection of H5N1 avian influenza virus from mosquitoes collected in an infected poultry farm in Thailand. Vector Borne Zoonotic Dis (2008) 0.81
Sustained deficiency of mitochondrial complex I activity during long periods of survival after seizures induced in immature rats by homocysteic acid. Neurochem Int (2009) 0.80
Segregation pattern and biochemical effect of the G3460A mtDNA mutation in 27 members of LHON family. J Neurol Sci (2004) 0.80
Succinimidyl oleate, established inhibitor of CD36/FAT translocase inhibits complex III of mitochondrial respiratory chain. Biochem Biophys Res Commun (2009) 0.80
TMEM70 protein - a novel ancillary factor of mammalian ATP synthase. Biochim Biophys Acta (2008) 0.80
Development of a human mitochondrial oligonucleotide microarray (h-MitoArray) and gene expression analysis of fibroblast cell lines from 13 patients with isolated F1Fo ATP synthase deficiency. BMC Genomics (2008) 0.79
Two components in pathogenic mechanism of mitochondrial ATPase deficiency: energy deprivation and ROS production. Exp Gerontol (2006) 0.79
A Russian family of Slavic origin carrying mitochondrial DNA with a 9-bp deletion in region V and a long C-stretch in D-loop. Mitochondrion (2002) 0.78
Evaluation of mitochondrial function in isolated rat hepatocytes and mitochondria during oxidative stress. Altern Lab Anim (2007) 0.78
Hepatitis C virus infection and genotypes in Antananarivo, Madagascar. J Med Virol (2007) 0.77
Mitochondrial membrane potential and ATP production in primary disorders of ATP synthase. Toxicol Mech Methods (2004) 0.77
Physiological oxygenation status is required for fully differentiated phenotype in kidney cortex proximal tubules. Am J Physiol Renal Physiol (2006) 0.77
Carnitine supplementation alleviates lipid metabolism derangements and protects against oxidative stress in non-obese hereditary hypertriglyceridemic rats. Appl Physiol Nutr Metab (2015) 0.77
Coenzyme Q releases the inhibitory effect of free fatty acids on mitochondrial glycerophosphate dehydrogenase. Acta Biochim Pol (2003) 0.77
Mitochondrial diseases and ATPase defects of nuclear origin. Biochim Biophys Acta (2004) 0.77
Mitochondrial Respiration in the Platelets of Patients with Alzheimer's Disease. Curr Alzheimer Res (2016) 0.77
Tissue specific sensitivity of mitochondrial permeability transition pore to Ca2+ ions. Acta Medica (Hradec Kralove) (2009) 0.76
Mitochondrial energy metabolism in very premature neonates. Biol Neonate (2002) 0.76
Differential expression of ATPAF1 and ATPAF2 genes encoding F(1)-ATPase assembly proteins in mouse tissues. FEBS Lett (2003) 0.76
Ultrastructural changes of mitochondria in the cultivated skin fibroblasts of patients with point mutations in mitochondrial DNA. Ultrastruct Pathol (2006) 0.76