Published in Cell Physiol Biochem on June 08, 2012
A kinase interacting protein (AKIP1) is a key regulator of cardiac stress. Proc Natl Acad Sci U S A (2013) 0.94
Optical spectroscopy in turbid media using an integrating sphere: mitochondrial chromophore analysis during metabolic transitions. Anal Biochem (2013) 0.81
The Role of Oxidative Stress in Myocardial Ischemia and Reperfusion Injury and Remodeling: Revisited. Oxid Med Cell Longev (2016) 0.78
Standardization of in vitro Cell-based Model for Renal Ischemia and Reperfusion Injury. Indian J Pharm Sci (2014) 0.76
Sensitivity of Interfibrillar and Subsarcolemmal Mitochondria to Cobalt Chloride-induced Oxidative Stress and Hydrogen Sulfide Treatment. Indian J Pharm Sci (2016) 0.75
Hydrogen sulfide post-conditioning preserves interfibrillar mitochondria of rat heart during ischemia reperfusion injury. Cell Stress Chaperones (2016) 0.75
Deleterious mutation in the mitochondrial arginyl-transfer RNA synthetase gene is associated with pontocerebellar hypoplasia. Am J Hum Genet (2007) 3.55
Control of pancreatic β cell regeneration by glucose metabolism. Cell Metab (2011) 3.35
Defective mitochondrial translation caused by a ribosomal protein (MRPS16) mutation. Ann Neurol (2004) 2.09
Deficiency of the ADP-forming succinyl-CoA synthase activity is associated with encephalomyopathy and mitochondrial DNA depletion. Am J Hum Genet (2005) 2.06
Lamina cribrosa thickness and spatial relationships between intraocular space and cerebrospinal fluid space in highly myopic eyes. Invest Ophthalmol Vis Sci (2004) 2.01
Distinct clinical phenotypes associated with a mutation in the mitochondrial translation elongation factor EFTs. Am J Hum Genet (2006) 1.97
Anatomic relationship between lamina cribrosa, intraocular space, and cerebrospinal fluid space. Invest Ophthalmol Vis Sci (2003) 1.79
Mutations in the fatty acid 2-hydroxylase gene are associated with leukodystrophy with spastic paraparesis and dystonia. Am J Hum Genet (2008) 1.71
Acute infantile liver failure due to mutations in the TRMU gene. Am J Hum Genet (2009) 1.61
Zinc-desferrioxamine attenuates retinal degeneration in the rd10 mouse model of retinitis pigmentosa. Free Radic Biol Med (2011) 1.59
The Thr224Asn mutation in the VPS45 gene is associated with the congenital neutropenia and primary myelofibrosis of infancy. Blood (2013) 1.55
Mutations in LPIN1 cause recurrent acute myoglobinuria in childhood. Am J Hum Genet (2008) 1.48
The potential of desferrioxamine-gallium as an anti-Pseudomonas therapeutic agent. Proc Natl Acad Sci U S A (2008) 1.41
Mice deficient in ribosomal protein S6 phosphorylation suffer from muscle weakness that reflects a growth defect and energy deficit. PLoS One (2009) 1.24
Early prenatal ventriculomegaly due to an AIFM1 mutation identified by linkage analysis and whole exome sequencing. Mol Genet Metab (2011) 1.20
Plasma antioxidant status and cell injury after severe physical exercise. Proc Natl Acad Sci U S A (2003) 1.15
FASTKD2 nonsense mutation in an infantile mitochondrial encephalomyopathy associated with cytochrome c oxidase deficiency. Am J Hum Genet (2008) 1.13
Ablation of ceramide synthase 2 causes chronic oxidative stress due to disruption of the mitochondrial respiratory chain. J Biol Chem (2013) 1.11
Mutation in mitochondrial ribosomal protein MRPS22 leads to Cornelia de Lange-like phenotype, brain abnormalities and hypertrophic cardiomyopathy. Eur J Hum Genet (2010) 1.07
The transgenic overexpression of alpha-synuclein and not its related pathology associates with complex I inhibition. J Biol Chem (2010) 1.05
The interplay between SUCLA2, SUCLG2, and mitochondrial DNA depletion. Biochim Biophys Acta (2011) 1.05
Exocrine pancreatic insufficiency, dyserythropoeitic anemia, and calvarial hyperostosis are caused by a mutation in the COX4I2 gene. Am J Hum Genet (2009) 1.05
Infantile mitochondrial hepatopathy is a cardinal feature of MEGDEL syndrome (3-methylglutaconic aciduria type IV with sensorineural deafness, encephalopathy and Leigh-like syndrome) caused by novel mutations in SERAC1. Am J Med Genet A (2013) 1.04
Familial neonatal isolated cardiomyopathy caused by a mutation in the flavoprotein subunit of succinate dehydrogenase. Eur J Hum Genet (2010) 1.04
Kinetic properties of mutant human thymidine kinase 2 suggest a mechanism for mitochondrial DNA depletion myopathy. J Biol Chem (2002) 1.04
Mitochondrial complex I deficiency caused by a deleterious NDUFA11 mutation. Ann Neurol (2008) 1.02
The effect of mutated mitochondrial ribosomal proteins S16 and S22 on the assembly of the small and large ribosomal subunits in human mitochondria. Mitochondrion (2008) 1.00
Mitochondrial complex III deficiency associated with a homozygous mutation in UQCRQ. Am J Hum Genet (2008) 0.99
Agenesis of corpus callosum and optic nerve hypoplasia due to mutations in SLC25A1 encoding the mitochondrial citrate transporter. J Med Genet (2013) 0.98
Antibiotic effects on mitochondrial translation and in patients with mitochondrial translational defects. Mitochondrion (2009) 0.97
Leukoencephalopathy with accumulated succinate is indicative of SDHAF1 related complex II deficiency. Orphanet J Rare Dis (2012) 0.96
Apoptosis-like death, an extreme SOS response in Escherichia coli. MBio (2014) 0.95
Diagnostic serum glycosylation profile in patients with intellectual disability as a result of MAN1B1 deficiency. Brain (2014) 0.95
l-arginine:glycine amidinotransferase (AGAT) deficiency: clinical presentation and response to treatment in two patients with a novel mutation. Mol Genet Metab (2010) 0.95
Roles of ferritin and iron in ischemic preconditioning of the heart. Mol Cell Biochem (2002) 0.94
NDUFS4 mutations cause Leigh syndrome with predominant brainstem involvement. Mol Genet Metab (2009) 0.94
Cardiac-targeted transgenic mutant mitochondrial enzymes: mtDNA defects, antiretroviral toxicity and cardiomyopathy. Cardiovasc Toxicol (2008) 0.93
Injury induced by chemical warfare agents: characterization and treatment of ocular tissues exposed to nitrogen mustard. Invest Ophthalmol Vis Sci (2003) 0.92
Screening for active small molecules in mitochondrial complex I deficient patient's fibroblasts, reveals AICAR as the most beneficial compound. PLoS One (2011) 0.92
Infantile cerebellar-retinal degeneration associated with a mutation in mitochondrial aconitase, ACO2. Am J Hum Genet (2012) 0.92
Treatment of ocular tissues exposed to nitrogen mustard: beneficial effect of zinc desferrioxamine combined with steroids. Invest Ophthalmol Vis Sci (2005) 0.91
HU-331, a novel cannabinoid-based anticancer topoisomerase II inhibitor. Mol Cancer Ther (2007) 0.90
Heart protection by ischemic preconditioning: a novel pathway initiated by iron and mediated by ferritin. J Mol Cell Cardiol (2008) 0.90
Depletion of the other genome-mitochondrial DNA depletion syndromes in humans. J Mol Med (Berl) (2002) 0.89
A novel thiol antioxidant that crosses the blood brain barrier protects dopaminergic neurons in experimental models of Parkinson's disease. Eur J Neurosci (2005) 0.89
Toward genotype phenotype correlations in GFM1 mutations. Mitochondrion (2011) 0.87
Methionine-centered redox cycle in organs of the aero-digestive tract of young and old rats. Biogerontology (2008) 0.86
TAT-mediated delivery of LAD restores pyruvate dehydrogenase complex activity in the mitochondria of patients with LAD deficiency. Mol Ther (2008) 0.86
The unique neuroradiology of complex I deficiency due to NDUFA12L defect. Mol Genet Metab (2008) 0.85
TMEM70 mutations are a common cause of nuclear encoded ATP synthase assembly defect: further delineation of a new syndrome. J Med Genet (2010) 0.85
Replacement of the C6ORF66 assembly factor (NDUFAF4) restores complex I activity in patient cells. Mol Med (2013) 0.85
Protection of a ceramide synthase 2 null mouse from drug-induced liver injury: role of gap junction dysfunction and connexin 32 mislocalization. J Biol Chem (2013) 0.84
Energy status determines the distinct biochemical and physiological behavior of interfibrillar and sub-sarcolemmal mitochondria. Biochem Biophys Res Commun (2012) 0.84
Ischemic preconditioning of the rat retina: protective role of ferritin. Free Radic Biol Med (2007) 0.84
2-Hydroxylated sphingomyelin profiles in cells from patients with mutated fatty acid 2-hydroxylase. Lipids Health Dis (2011) 0.82
Effect of para-aminobenzoic acid on the course of retinal degeneration in the rd10 mouse. J Ocul Pharmacol Ther (2009) 0.82
Myocardial metabolism altered by ischemic preconditioning and enflurane in off-pump coronary artery surgery. J Cardiothorac Vasc Anesth (2008) 0.82
Mutated NDUFS6 is the cause of fatal neonatal lactic acidemia in Caucasus Jews. Eur J Hum Genet (2009) 0.82
Nonylphenol ethoxylate plastic additives inhibit mitochondrial respiratory chain complex I. Clin Chem (2009) 0.81
Alteration in iron metabolism during retinal degeneration in rd10 mouse. Invest Ophthalmol Vis Sci (2008) 0.81
Ischemic preconditioning decreases the reperfusion-related formation of hydroxyl radicals in a rabbit model of regional myocardial ischemia and reperfusion: the role of K(ATP) channels. Free Radic Res (2005) 0.81
Leigh disease presenting in utero due to a novel missense mutation in the mitochondrial DNA-ND3. Mol Genet Metab (2010) 0.81
The effect of restraint stress on the normal colon and on intestinal inflammation in a model of experimental colitis. Dig Dis Sci (2007) 0.81
Prophylactic administration of topical glutamine enhances the capability of the rat colon to resist inflammatory damage. Dig Dis Sci (2004) 0.81
Aging is an organ-specific process: changes in homeostasis of iron and redox proteins in the rat. Age (Dordr) (2011) 0.80
Cardiac protection by preconditioning is generated via an iron-signal created by proteasomal degradation of iron proteins. PLoS One (2012) 0.80
Mitochondrial performance in heat acclimation--a lesson from ischemia/reperfusion and calcium overload insults in the heart. Am J Physiol Regul Integr Comp Physiol (2012) 0.80
The bitter fate of the sweet heart: impairment of iron homeostasis in diabetic heart leads to failure in myocardial protection by preconditioning. PLoS One (2013) 0.80
The effect of antiepileptic drugs on mitochondrial activity: a pilot study. J Child Neurol (2010) 0.80
Flumazenil mimics whereas midazolam abolishes ischemic preconditioning in a rabbit heart model of ischemia-reperfusion. Anesthesiology (2006) 0.80
alpha-Synuclein abnormalities in mouse models of peroxisome biogenesis disorders. J Neurosci Res (2010) 0.79
Elevated plasma citrulline: look for dihydrolipoamide dehydrogenase deficiency. Eur J Pediatr (2013) 0.79
Zinc-desferrioxamine reduces damage to lenses exposed to hyperbaric oxygen and has an ameliorative effect on catalase and Na, K-ATPase activities. Exp Eye Res (2006) 0.79
Desferrioxamine and zinc-desferrioxamine reduce lens oxidative damage. Exp Eye Res (2007) 0.79
IL-1β hampers glucose-stimulated insulin secretion in Cohen diabetic rat islets through mitochondrial cytochrome c oxidase inhibition by nitric oxide. Am J Physiol Endocrinol Metab (2014) 0.78
Dietary copper supplementation restores β-cell function of Cohen diabetic rats: a link between mitochondrial function and glucose-stimulated insulin secretion. Am J Physiol Endocrinol Metab (2013) 0.78
Diabetes and the heart: could the diabetic myocardium be protected by preconditioning? Redox Rep (2007) 0.78
Time window of nitroxide effect on myocardial ischemic-reperfusion injury potentiated by iron. Free Radic Biol Med (2002) 0.78
Human granulosa luteal cell oxidative phosphorylation function is not affected by age or ovarian response. Fertil Steril (2012) 0.77
Are there metallic traces in black extrinsic dental stain? Quintessence Int (2013) 0.77
Mitochondrial OXPHOS function is unaffected by chronic azithromycin treatment. J Cyst Fibros (2013) 0.77
Iron homeostasis and methionine-centred redox cycle in nasal polyposis. Free Radic Res (2010) 0.76
Use of amphoteric rinsing solution for treatment of ocular tissues exposed to nitrogen mustard. Acta Ophthalmol (2012) 0.75